Systemic lupus erythematosus in children. Lupus erythematosus in children is a complex autoimmune disease.

Due to lupus erythematosus, serious systemic damage to the whole organism (blood vessels, connective tissues, organs) occurs. Girls in puberty are more often exposed to this incurable disease. Only about 5% of cases are boys. The disease is very difficult to diagnose, because its manifestations are very similar to other childhood ailments.

Causes

Types of pathology

Acute

subacute

Chronic

Diagnostics

Preventive advice

Causes

Basically, they become a trigger for lupus erythematosus in children with increased sensitivity to various external factors. The impetus for the disease (but not the direct cause) can be:

solar exposure;
hypothermia;
stressful situations;
overwork;
physical and psychological trauma.

All these factors become especially significant during the period of hormonal changes in the body, its physiological allergization.

An important role in the development of lupus erythematosus is played by heredity. Indirectly, the genetic nature of the disease is evidenced by "family" cases of the disease, as well as cases of rheumatism, arthritis and other diffuse connective tissue pathologies that are common among relatives.

In children, lupus erythematosus accounts for 20% of all cases of morbidity. In young children, it happens in exceptional cases. CV can fully manifest itself by 9-10 years. Due to the genetic characteristics of the female body, lupus is more common in girls than in boys.

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Types of pathology

Lupus erythematosus can be of 3 types:

discoid lupus erythematosus;
disseminated;
systemic lupus erythematosus.

With disseminated CV, peripheral growth of foci is not observed. A disordered rash appears on the skin of the face or on the ears, chest, back. The surface layer of the skin atrophies. When the head is affected by lupus, it begins to go bald.

Note! The most dangerous form is systemic lupus erythematosus. It affects all organs and systems, and has a lot of manifestations.

Characteristic signs and symptoms

It is almost impossible to immediately determine that a child has lupus erythematosus. The onset of the disease proceeds as a lesion of one specific organ or system. Gradually, the inflammatory symptoms subside. Then other manifestations begin, which have signs of a completely different disease.

The following symptoms of lupus erythematosus should alert:

complaints of joint and muscle pain;
weakness;
fever;
red rash in the form of a butterfly on the cheeks and bridge of the nose;
red spots on the back, neck, head, chest;
increasing dystrophy;
inflammation of the lymph nodes;
ulcerative lesions of the mucous membranes of the nose and mouth;
nervousness and depression;
swelling of the hands and feet.

Forms of lupus erythematosus in children

The symptomatology of CV largely depends on the characteristics of its course. In this regard, there are 3 forms of the disease.

Has a progressive character. The child has:

decreased mobility;
fever;
severe headache;
general intoxication;
feeling of aching joints;
rash on the face like a "butterfly".

In the first months of CV, the kidneys are involved in the lesion process. Symptoms of kidney disease are added to the general clinical manifestations of the disease.

subacute

Most cases of subacute lupus erythematosus begin as polyarthritis. The child in turn has inflammation of several joints. A characteristic rash appears on the cheeks and bridge of the nose.

Other symptoms:

nephritis;
loss of appetite;
weight loss;
carditis;
polyserositis.

Chronic

This form of CV is the most difficult to diagnose. Occurs in 1/3 of cases. The disease first proceeds monosyndromic, that is, it has signs of damage to one organ. The clinical picture is blurred. Other organs and systems are involved in the process very slowly. Alternately, recurrences of articular syndrome or skin rashes appear. This process can take many years with long remissions. Unlike adults, CV in children often has an acute onset and a malignant course, sometimes it can lead to death.

Diagnostics

The diagnosis of lupus erythematosus can only be made in a hospital setting, when the child's symptoms of one disease or another cannot be treated. Therefore, many studies are assigned, the results of which can confirm the presence of CV. There are no separate tests for lupus erythematosus. The disease is diagnosed based on specific symptoms and laboratory tests.

Mandatory tests for CV:

biochemical and general blood test;
Analysis of urine;
detection of ANF, LE-cells and antibodies to DNA in high titers in the blood.

Sometimes even experienced doctors cannot determine the CV, and diagnose other diseases (rheumatism, nephritis, arthritis). And they can be manifestations of a more serious pathology - systemic lupus.

Methods and general rules of treatment

The disease is currently considered incurable. Therapy is aimed only at relieving symptoms and stopping the autoimmune and inflammatory process. A child with severe symptoms of a relapse of the disease should be treated in a hospital.

Corticosteroids are the first choice for treating lupus erythematosus:

Prednisolone;
Dexamethasone;
Urbazon and others.

Treatment with the maximum dose of corticosteroids should be continued for 1-2 months (with symptoms of nephrotic nephritis longer), until the clinical manifestations of lupus disappear. Gradually, the patient is transferred to a lower dosage of the drug as maintenance therapy. It may be several years. A sharp decrease or cancellation of the drug can lead to a relapse of the pathology.

In chronic lupus erythematosus without damage to the central nervous system, visceral organs, corticosteroids are not prescribed or used in minimal dosages. (1/2 mg/kg). The drug should be discontinued for stomach ulcers, diabetes, hypertension, kidney failure.

With lupus nephritis, Cyclophosphamide is prescribed. It is administered intravenously at a maximum dose once a month (15-20 mg/kg body weight) for 1-1.5 years. After that, 1 time in 3 months for another 1-1.5 years. In case of inefficiency of Cyclophosphamide, nephrotic syndrome is stopped with Cyclosporine (5 mg/kg). In the presence of severe complications after taking glucocorticoids, Azathioprine (1-2 mg/kg) is sometimes used to maintain remission of nephritis.

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Together with corticosteroids, the child is prescribed:

anticoagulants (Acenocoumarol, Heparin);
antihypertensive agents;
antibiotics;
antiplatelet agents.

A child with lupus erythematosus should be under the constant supervision and control of a specialist. In addition to drug therapy, one should adhere to a diet close to anti-ulcer (restrict carbohydrates, exclude extractive and juice products, enrich the menu with potassium salts and protein). The child's body should have enough vitamins, especially groups B and C.

If a child is ill with lupus erythematosus, then regular follow-up by a cardiorheumatologist should become secondary prevention to prevent relapses. He prescribes anti-relapse treatment that maintains remission and prevents possible exacerbations of CV.

Lupus erythematosus in children is much more severe than in adults, and is practically not cured. Therefore, it is very important to correctly determine the tactics of treatment, and strictly adhere to it. Thanks to the development of modern medicine, today the course of CV is easier, the number of relapses is decreasing.

Video. TV show "Live Healthy" about lupus erythematosus:

Systemic lupus erythematosus in children develops in the process of pathology of the immune system, which leads to imperfection of regulatory functions. There is a production of a large number of antibodies to their own cells. A complex inflammation of the body develops.

Causes

Signs of lupus erythematosus are observed with a shift at the hormonal level. Girls suffer from the disease more often than boys. In sick girls, there is an increased background of estrogenic activity. In boys, the cause is a decrease in testosterone and an increased background of estradiol.
The environment can trigger the symptoms of lupus erythematosus in a child. Solar radiation causes disease quite often.
The cause of children's illness is the intake of tetracycline drugs, sulfonamides, antiarrhythmic and anticonvulsant drugs after other diseases.
Lupus erythematosus sometimes becomes a complication of viral diseases.

Forms

Acute and subacute

The disease develops rapidly in an acute form and affects the internal organs of a sick child. In the subacute form, the disease occurs in waves with periods of remissions and exacerbations. Damage to the internal organs will join after 3 years from the onset of the disease.

Chronic

The chronic form is characterized by the duration of one symptom, for example, skin rashes or impaired hematopoiesis. After 5 years of this form of lupus erythematosus, the nervous system will suffer, and the kidneys will be affected.

Symptoms

In children, lupus erythematosus is more severe, in adulthood it is easier. The incidence is noted from the age of 9, and the age from 12 to 14 years becomes the peak. The clinical picture manifests itself with a high temperature rise - fever, with skin and joint syndromes.

The patient has increasing signs of dystrophy and symptoms of damage to internal organs, diffuse generalized vasculitis develops.

On the skin, we observe urticaria, erythema with exudate edema. Or infiltrates with necrotic ulcers or vesicles that leave marks, scars, or pigmentation. Infiltrates are localized in open areas of the body: chest, arms, face. In adults, a skin lesion in the form of a lupus butterfly occurs, it manifests itself in small areas and quickly disappears. Lupus is rare in children.

Lupus arthritis in children is characterized as the initial syndrome of lupus erythematosus, in which the joints are affected. With lupus arthritis, pain, weakness, and muscle compaction are manifested, which migrate throughout the body and cause edema, tissue infiltrates between the muscles. Joint syndrome is combined with myositis, myalgia.

In a child with systemic lupus erythematosus, serous membranes are affected due to bilateral pleurisy, pericarditis. Symptoms of visceral lesions will be characterized by carditis in an adult, in a child - myocarditis. Rarely, endocarditis occurs.

With lesions in the lungs, lupus pneumonitis occurs. Complaints: chest pain, cough without sputum, shortness of breath. Children have nephritis. In adolescent children, 10 percent of the disease begins with nephritis.

Lupus neurolupus speaks of a disease of the nervous system. It occurs in 50% of childhood lupus erythematosus. In the brain, in the subcortex, due to vascular thrombosis, the substance softens in foci. There are symptoms of a neurotic nature, dizziness, headache, sleep disturbances. Not rare cases of epilepsy.

Symptoms of damage to the gastrointestinal tract. Abdominal pain caused by pancreatitis. Repeated diarrhea, vomiting, nausea. The child will have an enlarged liver and spleen. Hematopoietic lesions - anemia, thrombocytopenia, lymphopenia, increased ESR and C-reactive protein. In acute, subacute forms, body temperature rises to 40 degrees. The child's condition is lethargic.

Diagnostics

To identify lupus erythematosus, an analysis of complaints and anamnesis is needed.

Identification of skin rashes, cough, pain in the joints and behind the sternum, shortness of breath, palpitations, increased blood pressure, swelling;
Examination of the skin of the face for the presence of rashes, redness, scales;
Dilated veins in the legs;
Inflammatory signs of the joints;
neurological disorders;
To detect pleurisy, the diagnosis of the respiratory organs is carried out on an X-ray machine;

The diagnosis is made in the presence of a typical picture of the disease and laboratory data from a blood test. Markers will be: antibodies to native double-stranded DNA, antinuclear factor, Antibodies with CM antigen, LE cells and lupus anticoagulant

Treatment

Treatment is carried out in a hospital.

With glomerulonephritis, rheumatism, a special table and bed rest are prescribed. Glucocorticosteroids are prescribed - Prednisolone. If the patient has lupus nephritis, cyclosporine A is prescribed, a course of 6-8 weeks;
Prescribing non-steroidal anti-inflammatory drugs such as Diclofenac, indomethacin;
It is important to improve blood microcirculation, so the treatment will be antithrombotic drugs - Trental;
Antiviral drugs are an integral part of the treatment, drugs - gammaferon, reaferon;
With lupus renal crisis, plasmapheresis is performed;
Osteoporosis is treated with calcium carbonate.

Complications

If the disease is diagnosed in time and treated in a timely manner, then 90% of sick children experience remission. In 10% of cases with lupus nephritis, renal failure, the prognosis of the disease will be unfavorable.

If the child does not receive timely treatment in an acute form, then a fatal outcome is inevitable. In the chronic form of lupus erythematosus, people can live for about 20 years.

With numerous lesions of various organs and the immune system, other diseases are also possible. When the scalp is affected, the follicles weaken, which can lead to complete or partial baldness.

Caution should be given to the child physiotherapy. Quartz is contraindicated for children and adults with lupus erythematosus. For children with a chronic course of the disease, prolonged sunbathing is contraindicated. Sick children with lupus erythematosus Transfusion therapy of plasma and blood is used only when indicated. You can use massage and exercise therapy, but only when the disease subsides.

After inpatient treatment, a sick child needs rehabilitation, which can be done in a sanatorium.

Prevention

Prevention will be the timely recognition of the child's illness and the immediate referral of the sick baby to the hospital. Prevention measures will be the prevention of exacerbations if the disease has already occurred and the extension of favorable periods. Strict control of a specialist during illness and observation after remission. Balanced diet and vitamin supplements. Abstinence from vaccinations that cause exacerbations of the disease. Contacting a doctor and treating infectious diseases in a timely manner.

Lupus erythematosus is not a sentence for childhood.

If treated on time and follow preventive measures, then unfavorable prognosis can be avoided. Stay healthy and take care of your children.

With the active development of modern medicine, an autoimmune disease called systemic lupus erythematosus (another name is Liebman-Sachs disease) is gaining momentum, which is increasingly crippling the ranks of children. The child's immune system produces antibodies that damage the DNA of perfectly healthy cells. This entails serious damage to the connective tissue, along with blood vessels throughout the body.

A dangerous and difficult-to-treat disease occurs most often in girls (only 5% of cases are boys) during puberty. Diagnosis is difficult, as the symptoms of the disease are very similar to other childhood ailments.

Symptoms

Recognizing the symptoms of lupus erythematosus in children can be very difficult even for an experienced doctor, not to mention parents. At the first manifestations of the disease, you can think of any other misfortune, but not about lupus. Its typical symptoms may be as follows:

fever with chills and profuse sweating;
dystrophy;
dermatitis, most often starting with a lesion of the bridge of the nose and cheeks and resembling a butterfly in appearance: edema, vesicles, necrotic ulcers, leaving behind scars or pigmentation;
the skin becomes thinner and becomes photosensitive;
allergic manifestations throughout the body: marbling, morbilliform rash, urticaria;
blood necrosis appears on the fingertips and palms;
hair loss up to baldness;
dystrophicity, fragility of the nail plates;
joint pain;
persistent and untreated stomatitis;
disturbances in the psyche of the child, who becomes nervous, irritable, capricious, unbalanced;
convulsions (in this case, you need to know: how to provide first aid for convulsions).

Such numerous symptoms of lupus erythematosus are explained by the fact that the disease progressively affects various organs of the child. What system of a small organism will fail - no one knows. The first signs of the disease may resemble a common allergy or dermatitis, which in fact will be just a consequence of the underlying disease - lupus. This leads to significant difficulties in diagnosing the disease.

Diagnostics

Lupus erythematosus is diagnosed in a child in a hospital, when numerous symptoms do not respond to any treatment, a lot of tests are prescribed, the results of which make the final diagnosis. If 4 of the following criteria are confirmed, doctors diagnose lupus:

Butterfly rash on cheeks and bridge of nose.
Stomatitis (presence of ulcers in the oral cavity).
Discoid rash on the skin (in the form of bright red spots all over the body).
Photosensitivity (skin sensitivity to sunlight).
Arthritis (ache due to inflammation) of several joints.
Damage to the heart and lungs: pleurisy, pericarditis.
Kidney diseases.
CNS problems: psychosis, seizures.
Hematological disorders (blood diseases).
Immunological indicators.

Lupus erythematosus with its symptoms can mislead even the most experienced doctor. When diagnosing rheumatism, arthritis, nephritis, capillary toxicosis, Werlhof's disease, sepsis, epilepsy, acute abdominal diseases, doctors often do not even realize that these are just the consequences and manifestations of a more serious and dangerous disease - systemic lupus. Problems also arise with the treatment of the disease.

Treatment

Treatment of systemic lupus erythematosus in children is carried out in a hospital and involves the use of the following therapy:

corticosteroids: prednisone, triamcinolone, dexamethasone, urbazone, etc.;
cytostatics; azathioprine, cyclophosphamide, chlorbutine;
immunosuppressants;
steroid-quinoline therapy;
a diet as close as possible to anti-ulcer: restriction of carbohydrates and fiber, the complete exclusion of juice dishes; base - proteins and potassium salts;
vitamin therapy (emphasis is on ascorbic acid and vitamins from subgroup B);
in the final stages of the disease - massage and physiotherapy exercises;
pulse therapy.

You should not take systemic lupus erythematosus in a child as a sentence. Modern medicine successfully copes with its progression, prolonging the life of children for decades. Death in childhood is rare, but the average life expectancy of people with this diagnosis is significantly shortened.

Systemic lupus erythematosus in children: treatment and symptoms

Systemic lupus erythematosus in children (lupus erythematosus disseminatus) is an immunocomplex disease characterized in children by rapid generalization of the pathological process, severe visceral manifestations, bright peripheral syndromes, and hyperimmune crises. The morphological basis of the disease is a universal capillaritis with a characteristic nuclear pathology and the deposition of immune complexes in the foci of tissue damage.

Systemic lupus erythematosus (SLE) has gone beyond the scope of a rare, casuistic pathology, but it still occurs in childhood much less frequently than acute rheumatism and rheumatoid arthritis.

Along with the systemic, there are also discoid and disseminated forms of lupus erythematosus, respectively, with single or multiple erythematous lesions on the skin without signs of damage to other organs and systems, without sharp immunological changes and lupus cells. Disseminated lupus erythematosus (DLE) occupies, as it were, an intermediate place between discoid and systemic, so those cases that occur with the presence of lupus cells can be attributed to SLE. However, all these forms should be considered as a manifestation of the same disease, and the possibility of transition from discoid or disseminated to systemic lupus apparently depends on the degree of sensitization of the organism, the strength of its protective reactions and the ability to localize the process.

Causes of the disease

Etiology. The cause of the disease is still unclear. In recent years, the question of the role of viral infection in the development of SLE has been discussed. A certain role is assigned to some drugs: antibiotics, sulfonamides, anticonvulsants and antihypertensives (hydralazine), as well as vaccines, gamma globulin. As a rule, they acquire the role of a trigger mechanism in individuals who have individual hypersensitivity to various exogenous factors. The impetus, but not the true cause of the disease, can also be such environmental influences as prolonged insolation, hypothermia, mental or physical trauma, etc. hormonal changes and physiological allergization of the body.

Modern studies have also established peculiar constitutional-family features of the body's reactivity that contribute to the development of SLE. Indirect evidence of hereditary predisposition to the disease are cases of "family" lupus, the development of SLE in identical twins, as well as an increased incidence of rheumatism, rheumatoid arthritis and other forms of diffuse connective tissue diseases among relatives of probands.

Development of the disease

Pathogenesis. Currently, the immunological theory of the development of SLE is generally accepted, according to which the activation and progression of the disease are due to the formation of immune complexes, including autoantibodies that can interact with the cell nucleus (antinuclear factor - ANF) or its individual components. A special pathogenetic role is attributed to autoantibodies to deoxyribonucleic acids (DNA) of the nuclei of the macroorganism's own cells. DNA itself is a weak antigen, but its ability to stimulate the production of antibodies is enhanced by the introduction of the virus into the cell. The interaction of antibody DNA with the cell nucleus leads to the latter's death and the release of nuclear detritus into the bloodstream. Fragments of nuclei found in tissues are the so-called hematoxylin bodies - a pathognomonic sign of SLE. The amorphous nuclear substance undergoes phagocytosis, which goes through the rosette stage: leukocytes accumulate around the nuclear detritus, then one of the leukocytes phagocytizes the detritus and turns into a lupus cell.

The intensity of the formation of immune complexes is indirectly judged by the content of serum complement or its components, assuming that the fall in the level of the latter reflects complement utilization in antigen-antibody reactions. A low complement level, along with an elevated titer of antibodies to DNA or ANF, is evidence of SLE activity.

The formation of immune complexes, consisting mainly of immunoglobulins G, less often M, as well as DNA antigen and complement, occurs in the bloodstream. The deposition of immune complexes on the basement membrane of the vessels of the microvasculature of various organs and systems leads to immune inflammation in them.

In addition, the associated, as a rule, disseminated intravascular coagulation syndrome contributes to tissue ischemia and hemorrhages in organs due to fibrin deposits and microthrombosis of capillaries, arterioles and venules. This syndrome is always secondary to the immunopathological process and modifies the clinical picture of the disease in its own way.

Along with the features of humoral immunity, a certain role in the pathogenesis of SLE is assigned to delayed-type hypersensitivity. It is detected by high sensitization of lymphocytes to DNA, as well as by other tests. At the same time selective depression of cellular immunity is observed. The number of suppressor T-lymphocytes in the peripheral blood is reduced, which predetermines the excessive production of antibodies by B-lymphocytes.

Despite the successful development of the immunological theory, today it is still impossible to answer the question of what is the beginning and root cause in the complex pathogenetic chain of SLE development. Apparently, viruses, and possibly other damaging agents (insolation, drugs, vaccines, etc.) and stressful situations, as well as the physiological restructuring of the body during puberty, can cause an unusual immunological response in a certain group of people. Therefore, all the peculiarities of immunopathological processes developing in SLE, including delayed and immediate type hypersensitivity, should be considered primarily in the light of the characteristics of the response of the macroorganism. In this regard, the pathogenetic role of congenital and acquired disorders of enzymatic processes and types of acetylation is currently being studied. The hypothesis of molecular mimicry is being intensively developed, and other aspects of predisposition to the disease are also being investigated.

Symptoms of systemic lupus erythematosus in children

clinical picture. Systemic lupus erythematosus in children affects mainly girls, as well as females in general; boys and men make up only 5-10% of the total number of patients. The age of maximum physiological activity, including puberty, is considered the most vulnerable. Nevertheless, SLE occurs occasionally among children in the first months and first years of life. The rise in morbidity among children begins at the age of 9, peaking at 12-14 years of age.

The pathological process is characterized by steady progression with possible, sometimes quite long, long-term remissions occurring under the influence of treatment or spontaneously. In the acute period, there is always a fever of the wrong type, sometimes taking on a hectic character with chills and profuse sweat. Characterized by dystrophy, often reaching cachexia, significant changes in the blood and signs of damage to various organs and systems. The latter can manifest themselves without a definite sequence, independently of one another, at different times from the onset of the disease, and in any combination.

Approximately 2/3 of patients have a typical skin lesion, manifested by exudative erythema with edema, infiltration with hyperkeratosis, often with a tendency to form vesicles and necrotic ulcers, leaving behind atrophic superficial scars or nested pigmentation. A combination of acute exudative and chronic discoid changes in the form of limited pink-red spots with whitish-gray scales and thinning of the skin, which starts from the center and gradually captures the entire focus, is very characteristic.

Localization of lupus dermatitis can be very diverse, but open areas of the skin are a favorite place: face, hands, chest. Erythema on the face with its outlines resembles a butterfly, the body of which is located on the nose, and the wings are on the cheeks. It can quickly disappear, appear incompletely, in separate parts. Attention is drawn to the increased photosensitivity of the skin in patients with lupus. Insolation is one of the most frequent factors provoking exacerbation of the pathological process.

On the skin of patients with SLE, there may also be non-specific allergic manifestations, such as bright marbling, urticaria, or a measles-like rash. Vascular disorders, DIC syndrome and thrombocytopenia can lead to the appearance of a hemorrhagic rash, the development of capillaritis with micronecrosis on the fingertips and on the palms; general dystrophy leads to dryness and pigmentation disorders.

Along with the skin, its appendages are also affected. Hair falls out intensely, which often ends in patchy baldness and even complete baldness. Nails become dystrophic, brittle, transverse striation appears. The process involves the mucous membranes of the lips, mouth, upper respiratory tract and genital organs.

One of the first and most frequent clinical signs of the disease is articular syndrome in the form of volatile arthralgia, acute or subacute arthritis and periarthritis with mild, sometimes transient, exudative phenomena. Both small and large joints are affected. Lupus arthritis is not progressive.

How does systemic lupus erythematosus progress in children?

Joint deformity due to periarticular changes develops in exceptionally rare cases, even with a long-term course of the disease. Radiographs usually show intact articular cartilage, osteoporosis of varying degrees.

Myalgia and myositis are often observed. The latter are accompanied by a decrease in muscle tone, general muscle weakness, up to complete immobility, atrophy, migrating local seals and muscle pain reaction. They are based on lymphoid infiltrates of the intermuscular tissue and fibrinoid necrosis of the walls of the arteries, accompanied by interstitial edema. It should be remembered that muscle weakness and atrophy sometimes develop due to general dystrophy and intoxication.

The defeat of the serous membranes is so common that, along with arthritis and dermatitis, serositis constitutes the so-called small triad, which is very characteristic of SLE. Pleurisy and pericarditis are especially often recognized in the clinic, but according to autopsy data, each of them is rarely isolated and is almost always combined with peritonitis, perihepatitis, or perisplenitis. Lupus serositis is characterized by ephemerality; in rare cases, it proceeds severely with a large accumulation of fluid in the cavities.

Of the visceral manifestations of SLE, carditis is the most common. All three membranes of the heart can be affected, but in children and adolescents, the phenomena of myocarditis dominate. With diffuse myocarditis, there is an expansion of the boundaries and a muffled heart tones, a moderately pronounced systolic murmur appears, and sometimes the heart rhythm is disturbed. Pronounced coronaritis is accompanied by pain in the region of the heart. The ECG almost constantly reveals signs of a violation of the regenerative processes of the myocardium (decrease, smoothness, deformation and inversion of the G wave, less often a shift in the ST interval). Possible violation of intraventricular, as well as intra-atrial conduction.

Radiologically with diffuse myocarditis, an increase in the size of the heart, smoothness of the cardiac arches, and a decrease in myocardial contractility can be noted. Acute heart failure rarely develops. In addition to myocarditis, myocardial dystrophy often occurs.

Lupus endocarditis is almost always associated with myocarditis; its lifetime diagnosis is difficult. Unlike septic and rheumatic, it is designated as atypical abacterial endocarditis Libman-Sachs(named after the researchers who first described its features). It is characterized by parietal localization, although at the same time there is involvement of valves in the process. Most often, the mitral valve is affected in isolation or in combination with the tricuspid and aortic valves. Endocarditis does not always have a clear reflection in the clinic and can only be a morphological finding, especially with moderate sclerotic changes in valves or parietal localization of the process. In some cases, auscultation and FCG reveal a distinct systolic murmur of an organic nature or there is a combination of "muscular" systolic murmur with a clear diastolic murmur. In modern conditions, lupus carditis in a significant part of cases is completely cured and rarely leads to the formation of an organic defect with hemodynamic disorders.

Lung involvement in the clinic is recognized less frequently than pleural involvement, and is characterized in most patients by poor physical findings. However, at autopsy, it is found in all cases. Often, undulating current lupus pneumonitis with thickening and focal fibrinoid necrosis of the alveolar septa, intraalveolar and interstitial edema, pneumosclerosis phenomena can lead to respiratory failure. The scarcity of clinical data is contrasted by the distinct severity of radiological changes. Most often, bilateral persistent deformation of the vascular-interstitial pattern is observed throughout the lung fields, sometimes detected even during the period of clinical remission. During exacerbations, multiple focal-like shadows of medium density with uneven contours appear, sometimes merging with each other, but rarely accompanied by a reaction from the roots of the lungs. An X-ray finding may be large infiltrates and discoid atelectasis in the lung tissue, occurring silently, without eosinophilia, with rapid dynamics and not leading to tissue breakdown. The X-ray picture is often supplemented by signs of pleural damage and high standing of the diaphragm due to diaphragmatitis, pleurodiaphragmatic adhesions and adhesions, decreased tone of the muscles of the intestine and diaphragm, etc.

Lupus pneumonitis at the time of exacerbation, it is not always easy to distinguish from secondary banal pneumonia, which is indicated by leukocytosis with a neutrophilic shift, x-ray data and the effect of antibiotics.

Lupus nephritis occupies a special place among other visceritis in SLE, showing relative resistance to treatment and often determining the outcome of the disease as a whole. The more severe the SLE, the more often the kidneys are affected. On average, lupus nephritis occurs in 2/3 of patients. Signs of it can appear at any time of the disease, but mainly in the first months and always in its active period. In the clinic, it can manifest itself in different ways:

a) in the form of the so-called latent nephritis with minimal urinary syndrome, without edema, arterial hypertension and functional disorders;

b) as a pronounced (manifest) nephritis without nephrotic syndrome, but with significant changes in the urine, shifts in functional parameters and extrarenal manifestations;

c) as nephrotic nephritis with severe urinary syndrome, edema, hypertension, hypercholesterolemia.

Most patients (excluding those with minimal kidney damage) in the active period of nephritis have arterial hypertension and hyperazotemia. Functional studies indicate that, along with a drop in glomerular filtration, there are dysfunctions of the tubular nephron and a decrease in effective renal plasma flow.

urinary syndrome, observed in all variants, includes proteinuria, the severity of which corresponds to the clinical form of nephritis, as well as erythrocyte- and leukocyturia. Pathology of the urinary sediment is nonspecific.

Morphological examination reveals both specific signs of lupus nephritis (thickening of the basement membranes - "wire loops", nuclear pathology in the form of hematoxylin bodies and karyorrhexis, fibrinoid changes, hyaline thrombi in the lumen of the glomerular capillaries), and changes in the type of membranous or mesangial glomerulonephritis . The study of nephrobiopsy specimens using histochemistry and electron microscopy helps to recognize monosyndromic variants of SLE that occur as an isolated renal process (nephritic "mask" of SLE).

The course of lupus nephritis in children and adolescents is usually chronic with periods of exacerbations and a tendency to progress, up to the development of renal failure. Approximately 10% of patients have a rapidly progressive course of nephritis with a fatal outcome from uremia in a short time. In 1/3 of patients, nephritis has a course complicated by eclampsia or acute renal failure. The development of a secondarily wrinkled kidney with symptoms of azotemic uremia is rare, since death occurs at earlier stages. In recent years, with timely and intensive treatment, it is increasingly possible to reduce the activity of nephritis, to give it the character of a chronic process with long periods of minimal activity (latent course) or complete clinical and laboratory remission.

Involvement in the pathological process of the nervous system is diagnosed in more than half of children with SLE; an organic lesion of the central nervous system is called neurolupus. At the same time, scattered foci of softening of the brain substance develop in the cortex and in the subcortical region, due to thrombovasculitis of small vessels. At the same time, patients often complain of headache, a feeling of heaviness in the head, dizziness, and sleep disturbances. The isolated defeat of peripheral nerves gives a pain syndrome and paresthesias. An objective examination reveals a variety of focal or diffuse neurological symptoms in the form of polyneuritis, sciatica, myeloradiculoneuritis, myelitis, encephalitis, encephalomyeloradiculoneuritis, etc.

In severe diffuse lesions of the nervous system with the development of hemorrhage, acute cerebral edema or serous leptomeningitis, encephalitic or meningoencephalitic syndrome, mental disorders develop, paresis and paralysis, aphasia, amnesia develop, there may be loss of consciousness, coma or soporous state with a serious threat to life. Lupus cerebrovasculitis may present with epilepsy or chorea.

As a result of organic damage to the central nervous system, patients may develop severe trophic disorders of the skin, subcutaneous tissue, usually located symmetrically, prone to rapid progression and the formation of extensive and deep necrosis, difficult to treat. Accession of a secondary infection easily leads to the development of sepsis.

It should be emphasized that neurolupus, along with lupus nephritis, is one of the most severe and prognostically unfavorable SLE syndromes, torpid to corticosteroid drugs.

Quite often there are symptoms of damage to the gastrointestinal tract. Sometimes an abdominal syndrome with a clinical picture of an acute abdomen can become the leading sign of SLE. These so-called gastrointestinal crises mimic any disease of the abdominal cavity, such as appendicitis, cholecystitis, peritonitis, intestinal obstruction, ulcerative colitis, dysentery and other intestinal infections.

The basis of the abdominal syndrome in SLE is most often widespread diffuse or focal vasculitis of the abdominal organs with possible thrombosis of small vessels, leading to damage to the intestinal walls - hemorrhages, sometimes even to heart attacks and necrosis, followed by perforation and the development of intestinal bleeding or fibro-purulent peritonitis. A symptom complex of malignant Crohn's disease (terminal ileitis) is possible. Abdominal pain can also be caused by perihepatitis, perisplenitis, pancreatitis.

Liver pathology with the development of lupus inflammatory-dystrophic changes proper (lupus-hepatitis) is relatively rare. In most cases, hepatomegaly reflects the involvement of the liver as an organ of the reticuloendothelium in the immunopathological process. Complaints may be due to overstretching of the capsule with a significant increase in the organ, biliary dyskinesia, or the presence of perihepatitis. The absence of functional disorders and rapid reversal in response to corticosteroid therapy indicate a predominantly reactive nature of hepatomegaly.

Damage to the hematopoietic organs and changes in peripheral blood are observed in all patients. The most characteristic symptom of SLE is leukopenia with a neutrophilic shift to myelocytes and promyelocytes. In the active period of the disease, the number of leukocytes decreases to 4 - 109 - 3 - 109 / l, and more severe leukopenia is possible. Sometimes it is replaced by leukocytosis, which reflects the influence of corticosteroid therapy or the addition of a banal infection. Autoimmune hemolytic anemia may develop with a drop in the number of erythrocytes to 1 - 1012 - 2 - 1012 / l, which has a serious prognostic value.

Along with leukopenia and anemia, thrombocytopenia is often observed. It differs little in clinical presentation from idiopathic thrombocytopenic purpura, as it also has an autoimmune origin. At the same time, a drop in the number of platelets often reflects the process of intravascular coagulation. Even with significant leukopenia, the bone marrow remains normoblastic. Its plasmatization with a corresponding increase in the number of plasma cells in the peripheral blood draws attention.

As a rule, the active period of SLE is characterized by increased ESR, reaching 50 - 70 - 90 mm / h. With the improvement of the condition, as well as under the influence of treatment, the ESR decreases markedly, during the period of remission it normalizes, although in many patients it remains within 16–25 mm/h. Signs of lupus include hyperproteinemia and dysproteinemia. During the period of maximum activity, the level of blood serum protein reaches 90 - PO g/l due to an increase in coarsely dispersed fractions: fibrinogen, gamma globulin, the content of which is 2 times higher than the age norm, reaching 30-40 rel.%. In addition, hypoalbuminemia, an increase in oti-globulins and especially a2-globulins, are observed.

Dysproteinemia and a significant increase in coarse proteins are the cause of positive sedimentary reactions and a number of serological tests (Vidal reaction, Paul-Bunnel, Wasserman, etc.). Along with this, in the active period of SLE, C-reactive protein, an increase in the diphenylamine reaction, the level of seromucoid, etc. are detected. None of them is specific for SLE, but, determined in dynamics, may be suitable for determining the degree of disease activity and selecting appropriate therapy .

During the period of remission, patients do not complain, lead an active lifestyle, and examination rarely reveals any signs of SLE. Sometimes it is possible to note changes in the blood, indicating the continuing tension of immunogenesis (increased levels of gamma globulin and immunoglobulins, the presence of antinuclear factor and antibodies to DNA, as well as a decrease in the complement content in blood serum, dysproteinemia, etc.).

Flow. Depending on the initial manifestations, an acute, subacute and chronic course of the disease is distinguished, and, by analogy with rheumatism, its high, moderate or low activity is distinguished. In the vast majority of children, SLE is acute and more malignant than in adults, with violent allergic reactions, high fever of the wrong type, early onset severe inflammatory-dystrophic changes in the internal organs, and sometimes ends in death in the first months from the onset of the disease.

Death in such cases most often occurs with symptoms of cardiopulmonary or renal failure against the background of intoxication and deep disturbances of homeostasis, hemocoagulation, water and electrolyte balance, as well as the addition of a secondary infection. Chronic SLE with a long multi-year presystemic period in children is rare. Usually in the coming months, less often - at the end of the first year or in the second year, a generalization of the pathological process occurs.

However, it should be remembered that often acute at the beginning and even rapidly developing SLE later acquires a chronic course with periods of long-term remissions. At the same time, the general development and growth of children occur relatively satisfactorily. At the same time, an acute malignant course with the development of a lupus crisis may also end with a chronically ongoing lupus process.

Complications. These include strokes and cerebral hemorrhages with paresis and paralysis, sepsis, phlebitis, trophic ulcers, aseptic necrosis of the femoral head.

Diagnosis and differential diagnosis

The most typical manifestation of the disease is considered to be a combination of lupus dermatitis with progressive dystrophy, anorexia, fever of the wrong type, arthropathies against the background of leukopenia, anemia, increased ESR and significant hypergammaglobulinemia. The clinical picture may be supplemented by lymphadenopathy, serositis, nephritis, endocarditis, pneumonitis. the diagnosis is greatly simplified if there is a lupus "butterfly". However, in children, as well as in adults, SLE for a certain time can be represented by a monosyndrome, which, when fading, can be replaced by another sign of the disease.

If we take into account the possibility of spontaneous and prolonged remissions, then such individual episodes are sometimes not linked together and systemic lupus erythematosus in children is not recognized for a long time.

Of particular diagnostic importance is the presence in the blood of patients with lupus cells (LE-cells), ANF and antibodies to DNA in high titers. The search for LE cells should be carried out repeatedly not only in the patient's blood, but also in the synovial, spinal, pleural, and pericardial fluids, if appropriate. If necessary, resort to a biopsy of the skin, muscles, lymph nodes, kidneys. Characteristic "butterfly" and dermatitis, the presence of lupus cells in an amount of at least 0.4% and ANF in high titer make the diagnosis of SLE reliable even with an asymptomatic clinic.

Most often, SLE has to be differentiated from rheumatism, rheumatoid arthritis, nephritis, capillary toxicosis, Werlhof's disease, sepsis, epilepsy, acute abdominal diseases, especially in the presence of monosyndromes.

Treatment of systemic lupus erythematosus in children

Each patient with severe clinical and laboratory signs of SLE activity must be treated in a hospital. The most effective therapeutic agents are corticosteroids: prednisolone (1 tablet - 5 mg), triamcinolone (1 tablet - 4 mg), dexamethasone (1 tablet - 0.5 mg), urbazone (1 tablet - 4 mg) and other analogues of prednisolone . Thanks to the use of corticosteroids, the rapid progression of the disease can stop, its activity can decrease, remission can occur, and the life of patients can be extended.

Signs of lupus erythematosus are observed with a shift at the hormonal level. Girls suffer from the disease more often than boys. In sick girls, there is an increased background of estrogenic activity. In boys, the cause is a decrease in testosterone and an increased background of estradiol.
The environment can trigger the symptoms of lupus erythematosus in a child. Solar radiation causes disease quite often.
The cause of children's illness is the intake of tetracycline drugs, sulfonamides, antiarrhythmic and anticonvulsant drugs after other diseases.
Lupus erythematosus sometimes becomes a complication of viral diseases.

Forms

Acute and subacute

Chronic

Symptoms

The patient has increasing signs of dystrophy and symptoms of damage to internal organs, diffuse generalized vasculitis develops.

Lupus arthritis – in children it is characteristic as the initial syndrome of lupus erythematosus, in which the joints are affected. With lupus arthritis, pain, weakness, and muscle compaction are manifested, which migrate throughout the body and cause edema, tissue infiltrates between the muscles. Joint syndrome is combined with myositis, myalgia.

Lupus neurolupus speaks of a disease of the nervous system. It occurs in 50% of childhood lupus erythematosus. In the brain, in the subcortex, due to vascular thrombosis, the substance softens in foci. There are symptoms of a neurotic nature, dizziness, headache, sleep disturbances. Not rare cases of epilepsy.

Diagnostics

To identify lupus erythematosus, an analysis of complaints and anamnesis is needed.

Identification of skin rashes, cough, pain in the joints and behind the sternum, shortness of breath, palpitations, increased blood pressure, swelling;
Examination of the skin of the face for the presence of rashes, redness, scales;
Dilated veins in the legs;
Inflammatory signs of the joints;
neurological disorders;
To detect pleurisy, the diagnosis of the respiratory organs is carried out on an X-ray machine;

Treatment

Treatment is carried out in a hospital.

With glomerulonephritis, rheumatism, a special table and bed rest are prescribed. Glucocorticosteroids are prescribed - Prednisolone. If the patient has lupus nephritis, cyclosporine A is prescribed, a course of 6-8 weeks;
Prescribing non-steroidal anti-inflammatory drugs such as Diclofenac, indomethacin;
It is important to improve blood microcirculation, so the treatment will be antithrombotic drugs - Trental;
Antiviral drugs are an integral part of the treatment, drugs - gammaferon, reaferon;
With lupus renal crisis, plasmapheresis is performed;
Osteoporosis is treated with calcium carbonate.

Complications

If the child does not receive timely treatment in an acute form, then a fatal outcome is inevitable. In the chronic form of lupus erythematosus, people can live for about 20 years.

With numerous lesions of various organs and the immune system, other diseases are also possible. When the scalp is affected, the follicles weaken, which can lead to complete or partial baldness.

After inpatient treatment, a sick child needs rehabilitation, which can be done in a sanatorium.

Prevention

Lupus erythematosus is not a sentence for childhood.

If treated on time and follow preventive measures, then unfavorable prognosis can be avoided. Stay healthy and take care of your children.

In the modern world, diseases that are associated with the immune system are increasingly common. One of them is lupus erythematosus (LE) in children. This is an autoimmune inflammation in which the immune system produces antibodies that attack the DNA of their own healthy cells. Due to lupus erythematosus, serious systemic damage to the whole organism (blood vessels, connective tissues, organs) occurs. Girls in puberty are more often exposed to this incurable disease. Only about 5% of cases are boys. The disease is very difficult to diagnose, because its manifestations are very similar to other childhood ailments.

Causes

Types of pathology

Acute

subacute

Chronic

Diagnostics

Preventive advice

Causes

There are many theories as to why children develop lupus. The disease has not yet been fully studied, so no one can name its exact causes. But most experts tend to consider this autoimmune disorder a viral infection. The influence of drugs on the state of the immune system (antibiotics, vaccines, gamma globulin) is also not excluded. Basically, they become a trigger for lupus erythematosus in children with increased sensitivity to various external factors. The impetus for the disease (but not the direct cause) can be:

solar exposure;
hypothermia;
stressful situations;
overwork;
physical and psychological trauma.

All these factors become especially significant during the period of hormonal changes in the body, its physiological allergization. An important role in the development of lupus erythematosus is played by heredity. Indirectly, the genetic nature of the disease is evidenced by "family" cases of the disease, as well as cases of rheumatism, arthritis and other diffuse connective tissue pathologies that are common among relatives. In children, lupus erythematosus accounts for 20% of all cases of morbidity. In young children, it happens in exceptional cases. CV can fully manifest itself by 9-10 years. Due to the genetic characteristics of the female body, lupus is more common in girls than in boys.

Find out the dosage and instructions for use of the drug Immunal for children. Effective methods for treating synechia in girls are described on this page.

Types of pathology

Lupus erythematosus can be of 3 types:

discoid lupus erythematosus;
disseminated;
systemic lupus erythematosus.

Discoid and disseminated forms are characterized by damage mainly to the surface of the skin. A rash appears on it in the face, neck, back, chest. With Discoid CV, these are pink and red spots that increase in size and transform into plaques with a red border. On the face, the rash looks like a butterfly. Hyperkeratosis is formed in the center of the plaques. Scales are difficult to remove. With disseminated CV, peripheral growth of foci is not observed. A disordered rash appears on the skin of the face or on the ears, chest, back. The surface layer of the skin atrophies. When the head is affected by lupus, it begins to go bald. Note! The most dangerous form is systemic lupus erythematosus. It affects all organs and systems, and has a lot of manifestations.

Characteristic signs and symptoms

complaints of joint and muscle pain;
weakness;
fever;
red rash in the form of a butterfly on the cheeks and bridge of the nose;
red spots on the back, neck, head, chest;
increasing dystrophy;
inflammation of the lymph nodes;
ulcerative lesions of the mucous membranes of the nose and mouth;
nervousness and depression;
swelling of the hands and feet.

Forms of lupus erythematosus in children

The symptomatology of CV largely depends on the characteristics of its course. In this regard, there are 3 forms of the disease.

Has a progressive character. The child has:

decreased mobility;
fever;
severe headache;
general intoxication;
feeling of aching joints;
rash on the face like a "butterfly".

In the first months of CV, the kidneys are involved in the lesion process. Symptoms of kidney disease are added to the general clinical manifestations of the disease.

subacute

Most cases of subacute lupus erythematosus begin as polyarthritis. The child in turn has inflammation of several joints. A characteristic rash appears on the cheeks and bridge of the nose. Other symptoms:

nephritis;
loss of appetite;
weight loss;
carditis;
polyserositis.

Chronic

Diagnostics

Mandatory tests for CV:

biochemical and general blood test;
Analysis of urine;
detection of ANF, LE-cells and antibodies to DNA in high titers in the blood.

Methods and general rules of treatment

Prednisolone;
Dexamethasone;
Urbazon and others.

Corticosteroids stop the active progress of CV, reduce its activity. They contribute to the rapid onset of remission. The dosage of drugs is determined by the degree of activity of the process, and not by the age of the patient. With lupus activity of 2-3 degrees, in which internal organs are affected, the daily dose of Prednisolone is 1-1.5 mg / kg of weight. If there are symptoms of nephritis, neurolupus, pancarditis, the dosage can be increased. In some cases, 1000 mg of a corticosteroid is administered simultaneously intravenously for 3 days, then they switch to internal medication in medium dosages. Treatment with the maximum dose of corticosteroids should be continued for 1-2 months (with symptoms of nephrotic nephritis longer), until the clinical manifestations of lupus disappear. Gradually, the patient is transferred to a lower dosage of the drug as maintenance therapy. It may be several years. A sharp decrease or cancellation of the drug can lead to a relapse of the pathology. In chronic lupus erythematosus without damage to the central nervous system, visceral organs, corticosteroids are not prescribed or used in minimal dosages. (1/2 mg/kg). The drug should be discontinued for stomach ulcers, diabetes, hypertension, kidney failure. With lupus nephritis, Cyclophosphamide is prescribed. It is administered intravenously at a maximum dose once a month (15-20 mg/kg body weight) for 1-1.5 years. After that, 1 time in 3 months for another 1-1.5 years. In case of inefficiency of Cyclophosphamide, nephrotic syndrome is stopped with Cyclosporine (5 mg/kg). In the presence of severe complications after taking glucocorticoids, Azathioprine (1-2 mg/kg) is sometimes used to maintain remission of nephritis.

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Together with corticosteroids, the child is prescribed:

anticoagulants (Acenocoumarol, Heparin);
antihypertensive agents;
antibiotics;
antiplatelet agents.

The primary preventive measures should be the overall health of children, as well as identifying among them groups with an increased risk of getting sick. This should include children with symptoms of lupus diathesis and those with a family history of rheumatic disease. Such children must strictly observe the rules for prescribing and using medicines, vaccinations, hardening measures. If a child is ill with lupus erythematosus, then regular follow-up by a cardiorheumatologist should become secondary prevention to prevent relapses. He prescribes anti-relapse treatment that maintains remission and prevents possible exacerbations of CV. Lupus erythematosus in children is much more severe than in adults, and is practically not cured. Therefore, it is very important to correctly determine the tactics of treatment, and strictly adhere to it. Thanks to the development of modern medicine, today the course of CV is easier, the number of relapses is decreasing. Video. TV show "Live Healthy" about lupus erythematosus:

Symptoms

fever with chills and profuse sweating;
dystrophy;
dermatitis, most often starting with a lesion of the bridge of the nose and cheeks and resembling a butterfly in appearance: edema, vesicles, necrotic ulcers, leaving behind scars or pigmentation;
the skin becomes thinner and becomes photosensitive;
allergic manifestations throughout the body: marbling, morbilliform rash, urticaria;
blood necrosis appears on the fingertips and palms;
hair loss up to baldness;
dystrophicity, fragility of the nail plates;
joint pain;
persistent and untreated stomatitis;
disturbances in the psyche of the child, who becomes nervous, irritable, capricious, unbalanced;
convulsions (in this case, you need to know: how to provide first aid for convulsions).

Diagnostics

Butterfly rash on cheeks and bridge of nose.
Stomatitis (presence of ulcers in the oral cavity).
Discoid rash on the skin (in the form of bright red spots all over the body).
Photosensitivity (skin sensitivity to sunlight).
Arthritis (ache due to inflammation) of several joints.
Damage to the heart and lungs: pleurisy, pericarditis.
Kidney diseases.
CNS problems: psychosis, seizures.
Hematological disorders (blood diseases).
Immunological indicators.

Treatment

Treatment of systemic lupus erythematosus in children is carried out in a hospital and involves the use of the following therapy:

corticosteroids: prednisone, triamcinolone, dexamethasone, urbazone, etc.;
cytostatics; azathioprine, cyclophosphamide, chlorbutine;
immunosuppressants;
steroid-quinoline therapy;
a diet as close as possible to anti-ulcer: restriction of carbohydrates and fiber, the complete exclusion of juice dishes; base - proteins and potassium salts;
vitamin therapy (emphasis is on ascorbic acid and vitamins from subgroup B);
in the final stages of the disease - massage and physiotherapy exercises;
pulse therapy.

Systemic lupus erythematosus is an autoimmune disease damaging DNA cells and blood vessels. This is a serious disease that requires complex therapy. According to statistics, lupus erythematosus affects mainly girls in adolescence, when they begin processes transition to adulthood. Among all cases of systemic lupus erythematosus in children, only 5% of patients are boys.

Read more about the symptoms and treatment of rheumatoid arthritis in children here.

Description and characteristics

It is a diffuse disease of human connective tissues characterized by immune lesion blood vessels and cell DNA produced by antibodies produced by the immune system. That is, the immune system perceives the elements of connective tissue and its derivatives as foreign bodies, and then attacks them, causing inflammation and other processes. The official name of the disease is Libman-Sachs disease, in honor of the two doctors who first described the disease.

Its unofficial name - lupus erythematosus - the disease received due to its characteristic rash on the face, which resembles a bite mark from a cleft palate in the shape of a butterfly.

This disease is poorly understood, and its true causes are still unknown like most autoimmune diseases. However, the vast majority of specialists are inclined to the version of the viral origin of the pathology, although variants of the body's reaction to taking certain drugs that affect the state of the immune system are not ruled out, since the number of cases of the disease is growing in parallel with progress in medicine. Currently known Several causative factors:

transferred stress;
severe hypothermia;
frequent physical and emotional overwork;
solar exposure;
viral infection;
taking medications;
hormonal changes.

Most often, lupus occurs during adolescence when major hormonal changes occur in the human body.

In most cases, the disease appears in girls with an increase in the level of estrogen - the female hormone, in boys the cause is an increase in estradiol and a decrease in testosterone.

This disease occurs in 3 forms:

Acute- the disease occurs suddenly and develops rapidly, affecting the internal organs and causing a number of painful symptoms: aching joints, fever, severe headaches, a rash on the face.
subacute- the disease appears in waves in the form of periods of remission and exacerbation. The defeat of the internal organs begins in 2-3 years. It has its own special symptoms: loss of appetite, kidney failure, polyserositis, carditis.
Chronic– there is a slow defeat of the organs one by one. The clinical picture is unclear. At first, there may be a rash and problems with one of the organs, which does not raise suspicions of lupus, since other organs and systems are normal. Then the disease affects another organ, and so on. This can last for years, with periods of relief. The most difficult form of the disease to diagnose.

How is the operation of Hirschsprung's disease in children? Learn about it from our article.

Systemic lupus erythematosus has a number of symptoms similar to other diseases. Moreover, each individual case has its own characteristics. Highlight the most common symptoms for all cases:

a rash on the face in the form of red spots in the shape of a butterfly;
fever;
chills;
allergic manifestations on the skin (urticaria, prickly heat);
necrosis of blood vessels at the fingertips (pads);
nail dystrophy;
dermatitis of various forms;
hair loss, baldness;
stomatitis;
mental disorder;
thinning of the skin and their increased sensitivity to sunlight;
convulsions;
general dystrophy.

Symptoms of lupus erythematosus in children - photo: The variety of symptoms is explained by the fact that the disease can affect various organs and systems of the human body. The main and very first symptom is a rash. and what happens next is unknown.

To make a diagnosis of systemic lupus erythematosus, you must have at least 4-5 of the symptoms described above. Laboratory studies do not give results.

Treatment of systemic lupus erythematosus involves complex therapy with medications in a hospital setting. For this, the following types of drugs are used:

Glucocorticosteroids- steroid hormones that suppress the activity of the immune system and inhibit the production of antibodies, which allows you to remove inflammatory processes (Prednisolone, Urbazone, Dexamethasone, Triamcinolone).
Cytostatics- prevent rapid cell division, which inhibits the development of lupus (Chlorbutin, Cyclophosphamide, Cyclophosphamide, Azathioprine).
TNF-α blockers- drugs that suppress the activity of antibodies (Adalimumab, Infliximab, Etanercept).
Anticoagulants- prevent the formation of blood clots (Heparin, Acenocoumarol).
Antibiotics- are prescribed to destroy the infection that may accompany lupus during treatment, since the immune system is suppressed, which makes the child vulnerable to any pathogens.
Non-steroidal anti-inflammatory means - relieve inflammation and pain syndrome (Indomethacin, Diclofenac).

As an additional therapy, completely different drugs can be prescribed, since the disease can affect any organ. The choice of drugs is carried out by the attending physician, depending on the clinical picture. In addition, the patient can undergo special procedures:

plasmapheresis– collection and purification of blood from toxins;
hemosorption– extrarenal blood purification from hydrophobic toxic substances;
cryoplasmosorption– regulation and purification of blood plasma composition.

At the final stage, the patient is prescribed vitamin complexes to restore depressed immunity, as well as prescribe massage and exercise therapy for the rehabilitation of lost body functions.

With timely medical care, the prognosis favorable in 90% of cases.

Fatal outcomes are rare, mainly from renal failure or secondary infection as a complication after immune suppression and the onset of remission. Among possible complications of the disease the most common are:

hemorrhage in the brain;
nephritis;
complete or partial paralysis;
phlebitis;
sepsis;
necrosis;
trophic ulcers;
chronic dermatitis.

Systemic lupus erythematosus is difficult to diagnose and treat. It is impossible to defend against it or prevent its occurrence. It is a poorly understood disease dangerous to life. It is only important to study the possible symptoms of this disease in order to consult a doctor in time for help. Otherwise, in the absence of medical assistance possible death or serious complications which will have an impact on the quality of the entire subsequent life of the child.

How to treat acute balanoposthitis in a boy? Find out the answer right now.

You can learn about the dangers of systemic lupus erythematosus for children from the video:

Systemic lupus erythematosus in children: treatment and symptoms

Systemic lupus erythematosus (SLE) has gone beyond the scope of a rare, casuistic pathology, but it still occurs in childhood much less frequently than acute rheumatism and rheumatoid arthritis.

Causes of the disease

Development of the disease

Symptoms of systemic lupus erythematosus in children

How does systemic lupus erythematosus progress in children?

a) in the form of the so-called latent nephritis with minimal urinary syndrome, without edema, arterial hypertension and functional disorders;

b) as a pronounced (manifest) nephritis without nephrotic syndrome, but with significant changes in the urine, shifts in functional parameters and extrarenal manifestations;

c) as nephrotic nephritis with severe urinary syndrome, edema, hypertension, hypercholesterolemia.

It should be emphasized that neurolupus, along with lupus nephritis, is one of the most severe and prognostically unfavorable SLE syndromes, torpid to corticosteroid drugs.

Complications. These include strokes and cerebral hemorrhages with paresis and paralysis, sepsis, phlebitis, trophic ulcers, aseptic necrosis of the femoral head.

Diagnosis and differential diagnosis

Treatment of systemic lupus erythematosus in children

Systemic lupus erythematosus (SLE)- a chronic autoimmune disease caused by a malfunction of immune mechanisms with the formation of damaging antibodies to one's own cells and tissues. SLE is characterized by damage to the joints, skin, blood vessels and various organs (kidneys, heart, etc.).

The cause and mechanisms of the development of the disease

The cause of the disease has not been elucidated. It is assumed that the trigger mechanism for the development of the disease are viruses (RNA and retroviruses). In addition, people have a genetic predisposition to SLE. Women get sick 10 times more often, which is associated with the peculiarities of their hormonal system (high concentration of estrogen in the blood). The protective effect of male sex hormones (androgens) with respect to SLE has been proven. Factors that can cause the development of the disease can be a viral, bacterial infection, medications.

The basis of the mechanisms of the disease is a violation of the functions of immune cells (T and B - lymphocytes), which is accompanied by excessive formation of antibodies to the body's own cells. As a result of excessive and uncontrolled production of antibodies, specific complexes are formed that circulate throughout the body. Circulating immune complexes (CIC) settle in the skin, kidneys, on the serous membranes of internal organs (heart, lungs, etc.) causing inflammatory reactions.

Symptoms of the disease

SLE is characterized by a wide range of symptoms. The disease proceeds with exacerbations and remissions. The onset of the disease can be both lightning fast and gradual.
General symptoms

Fatigue
Weight loss
Temperature
Decreased performance
Fast fatiguability

Damage to the musculoskeletal system

Arthritis - inflammation of the joints
- Occurs in 90% of cases, non-erosive, non-deforming, joints of the fingers, wrists, knee joints are more often affected.
Osteoporosis - decreased bone density
- As a result of inflammation or treatment with hormonal drugs (corticosteroids).

Muscle pain (15-64% of cases), muscle inflammation (5-11%), muscle weakness (5-10%)

Mucosal and skin lesions

Skin lesions at the onset of the disease appear only in 20-25% of patients, in 60-70% of patients they occur later, in 10-15% of the skin manifestations of the disease do not occur at all. Skin changes appear on areas of the body exposed to the sun: face, neck, shoulders. Lesions have the appearance of erythema (reddish plaques with peeling), dilated capillaries along the edges, areas with excess or lack of pigment. On the face, such changes resemble the appearance of a butterfly, as the back of the nose and cheeks are affected.
Hair loss (alopecia) is rare, usually affecting the temporal region. Hair falls out in a limited area.
Increased skin sensitivity to sunlight (photosensitivity) occurs in 30-60% of patients.
Mucosal involvement occurs in 25% of cases.
- Redness, decreased pigmentation, malnutrition of the tissues of the lips (cheilitis)
- Small punctate hemorrhages, ulcerative lesions of the oral mucosa

Respiratory damage

Respiratory system lesions in SLE are diagnosed in 65% of cases. Pulmonary pathology can develop both acutely and gradually with various complications. The most common manifestation of damage to the pulmonary system is inflammation of the membrane covering the lungs (pleurisy). It is characterized by pain in the chest, shortness of breath. SLE can also cause the development of lupus pneumonia (lupus pneumonitis), characterized by: shortness of breath, cough with bloody sputum. SLE often affects the vessels of the lungs, leading to pulmonary hypertension. Against the background of SLE, infectious processes in the lungs often develop, and it is also possible to develop a serious condition such as blockage of the pulmonary artery by a thrombus (pulmonary embolism).

Damage to the cardiovascular system

SLE can affect all structures of the heart, the outer shell (pericardium), the inner layer (endocardium), directly the heart muscle (myocardium), valves and coronary vessels. The most common is the pericardium (pericarditis).

Pericarditis is an inflammation of the serous membranes that cover the heart muscle.

Manifestations: the main symptom is dull pain in the sternum. Pericarditis (exudative) is characterized by the formation of fluid in the pericardial cavity, with SLE, the accumulation of fluid is small, and the entire inflammation process usually lasts no more than 1-2 weeks.

Myocarditis is inflammation of the heart muscle.

Manifestations: heart rhythm disturbances, impaired conduction of a nerve impulse, acute or chronic heart failure.

The defeat of the valves of the heart, the mitral and aortic valves are more often affected.
Damage to the coronary vessels can lead to myocardial infarction, which can also develop in young patients with SLE.
Damage to the inner lining of blood vessels (endothelium) increases the risk of atherosclerosis. Peripheral vascular disease is manifested by:
- Livedo reticularis (blue spots on the skin creating a grid pattern)
- Lupus panniculitis (subcutaneous nodules, often painful, may ulcerate)
- Thrombosis of the vessels of the extremities and internal organs

Kidney damage

Most often in SLE, the kidneys are affected, in 50% of patients lesions of the renal apparatus are determined. A frequent symptom is the presence of protein in the urine (proteinuria), erythrocytes and cylinders are usually not detected at the onset of the disease. The main manifestations of kidney damage in SLE are: proliferative glomerulonephritis and mebran nephritis, which is manifested by nephrotic syndrome (proteins in the urine are more than 3.5 g / day, a decrease in protein in the blood, edema).

Damage to the central nervous system

It is assumed that CNS disorders are caused by damage to the cerebral vessels, as well as the formation of antibodies to neurons, to cells responsible for protecting and nourishing neurons (glial cells), and to immune cells (lymphocytes).
The main manifestations of damage to the nervous structures and blood vessels of the brain:

Headache and migraine, the most common symptoms in SLE
Irritability, depression - rare
Psychoses: paranoia or hallucinations
brain stroke
Chorea, parkinsonism - rare
Myelopathy, neuropathy and other disorders of the formation of nerve sheaths (myelin)
Mononeuritis, polyneuritis, aseptic meningitis

Digestive tract injury

Clinical lesions of the digestive tract are diagnosed in 20% of patients with SLE.

Damage to the esophagus, violation of the act of swallowing, expansion of the esophagus occurs in 5% of cases
Ulcers of the stomach and 12th intestine are caused both by the disease itself and by the side effects of treatment.
Abdominal pain as a manifestation of SLE, and can also be caused by pancreatitis, inflammation of the intestinal vessels, intestinal infarction
Nausea, abdominal discomfort, indigestion

Hypochromic normocytic anemia occurs in 50% of patients, the severity depends on the activity of SLE. Hemolytic anemia is rare in SLE.
Leukopenia is a decrease in white blood cells. It is caused by a decrease in lymphocytes and granulocytes (neutrophils, eosinophils, basophils).
Thrombocytopenia is a decrease in platelets in the blood. It occurs in 25% of cases, caused by the formation of antibodies against platelets, as well as antibodies to phospholipids (fats that make up cell membranes).

Also, in 50% of patients with SLE, enlarged lymph nodes are determined, in 90% of patients, an entrained spleen (splenomegaly) is diagnosed.

Diagnosis of SLE

Diagnosis of SLE is based on data from the clinical manifestations of the disease, as well as data from laboratory and instrumental studies. The American College of Rheumatology has developed special criteria by which it is possible to make a diagnosis - systemic lupus erythematosus.

Criteria for the diagnosis of systemic lupus erythematosus

The diagnosis of SLE is made if at least 4 out of 11 criteria are present.

Arthritis	Characteristic: without erosion, peripheral, manifested by pain, swelling, accumulation of insignificant fluid in the joint cavity
discoid rashes	Red in color, oval, round or annular in shape, plaques with uneven contours on their surface are scales, dilated capillaries nearby, scales are difficult to separate. Untreated lesions leave scars.
Mucosal lesions	The oral mucosa or nasopharyngeal mucosa is affected in the form of ulcerations. Usually painless.
photosensitivity	Increased sensitivity to sunlight. As a result of exposure to sunlight, a rash appears on the skin.
Rash on back of nose and cheeks	Specific rash in the form of a butterfly
Kidney damage	Permanent loss of protein in the urine 0.5 g/day, excretion of cellular casts
Damage to the serous membranes	Pleurisy is an inflammation of the membranes of the lungs. It is manifested by pain in the chest, aggravated by inhalation. Pericarditis - inflammation of the lining of the heart
CNS lesion	Convulsions, Psychosis - in the absence of drugs that can provoke them or metabolic disorders (uremia, etc.)
Changes in the blood system	Hemolytic anemia Reduction of leukocytes less than 4000 cells / ml Reduction of lymphocytes less than 1500 cells / ml Decrease in platelets less than 150 10 9 /l
Changes in the immune system	Altered amount of anti-DNA antibodies Presence of cardiolipin antibodies Antinuclear antibodies anti-Sm
Increasing the number of specific antibodies	Elevated anti-nuclear antibodies (ANA)

The degree of disease activity is determined by special SLEDAI indices ( Systemic lupus erythematosus disease activity index). The disease activity index includes 24 parameters and reflects the state of 9 systems and organs, expressed in points that are summarized. Maximum 105 points, which corresponds to very high disease activity.

Disease activity indices bySLEDAI

Manifestations	Description	Punctuation
Pseudo-epileptic seizure(development of convulsions without loss of consciousness)	It is necessary to exclude metabolic disorders, infections, medications that could provoke it.	8
psychoses	Violation of the ability to perform actions in the usual mode, impaired perception of reality, hallucinations, decreased associative thinking, disorganized behavior.	8
Organic changes in the brain	Changes in logical thinking, orientation in space is disturbed, memory, intelligence, concentration, incoherent speech, insomnia or drowsiness are reduced.	8
Eye disorders	Inflammation of the optic nerve, excluding arterial hypertension.	8
Damage to the cranial nerves	Damage to the cranial nerves revealed for the first time.
Headache	Severe, persistent, may be migraineous, not responding to narcotic analgesics	8
Cerebral circulatory disorders	First detected, excluding the consequences of atherosclerosis	8
Vasculitis-(vascular damage)	Ulcers, gangrene of the extremities, painful knots on the fingers	8
Arthritis- (inflammation of the joints)	Damage to more than 2 joints with signs of inflammation and swelling.	4
Myositis- (inflammation of skeletal muscles)	Muscle pain, weakness with confirmation of instrumental studies	4
Cylinders in the urine	Hyaline, granular, erythrocyte	4
erythrocytes in urine	More than 5 red blood cells in the field of view, exclude other pathologies	4
Protein in the urine	More than 150 mg per day	4
Leukocytes in urine	More than 5 white blood cells in the field of view, excluding infections	4
Skin lesions	Inflammatory damage	2
Hair loss	Enlargement of lesions or complete hair loss	2
Mucosal ulcers	Ulcers on the mucous membranes and on the nose	2
Pleurisy- (inflammation of the membranes of the lungs)	Chest pain, pleural thickening	2
Pericarditis-( inflammation of the lining of the heart)	Detected on ECG, echocardiography	2
Decreased compliment	Decreased C3 or C4	2
AntiDNA	Positively	2
Temperature	More than 38 degrees C, excluding infections	1
Decrease in blood platelets	Less than 150 10 9 /l, excluding medicines	1
Decrease in white blood cells	Less than 4.0 10 9 /l, excluding medicines	1

Light activity: 1-5 points
Moderate activity: 6-10 points
High activity: 11-20 points
Very high activity: more than 20 points

Diagnostic tests used to detect SLE

ANA- screening test, specific antibodies to cell nuclei are determined, is determined in 95% of patients, does not confirm the diagnosis in the absence of clinical manifestations of systemic lupus erythematosus
Anti DNA– antibodies to DNA, determined in 50% of patients, the level of these antibodies reflects the activity of the disease
Anti-sm- specific antibodies to the Smith antigen, which is part of short RNA, are detected in 30-40% of cases
Anti-SSA or Anti-SSB, antibodies to specific proteins located in the cell nucleus, are present in 55% of patients with systemic lupus erythematosus, are not specific for SLE, and are also detected in other connective tissue diseases
Anticardiolipin - antibodies to mitochondrial membranes (energy station of cells)
Antihistones- antibodies against proteins necessary for packaging DNA into chromosomes, characteristic of drug-induced SLE.

Other laboratory tests

Markers of inflammation
- ESR - increased
- C - reactive protein, elevated

Compliment level lowered
- C3 and C4 are reduced as a result of excessive formation of immune complexes
- Some people are born with reduced compliment levels, a predisposing factor for developing SLE.

The compliment system is a group of proteins (C1, C3, C4, etc.) involved in the body's immune response.

General blood analysis
- Possible decrease in red blood cells, white blood cells, lymphocytes, platelets

Analysis of urine
- Protein in the urine (proteinuria)
- Red blood cells in the urine (hematuria)
- Casts in the urine (cylindruria)
- White blood cells in urine (pyuria)

Blood chemistry
- Creatinine - an increase indicates kidney damage
- ALAT, ASAT - an increase indicates liver damage
- Creatine kinase - increases with damage to the muscular apparatus

Instrumental research methods

X-ray of the joints

Minor changes are detected, no erosion

X-ray and computed tomography of the chest

Reveal: damage to the pleura (pleurisy), lupus pneumonia, pulmonary embolism.

Nuclear magnetic resonance and angiography

CNS damage, vasculitis, stroke and other nonspecific changes are detected.

echocardiography

They will allow you to determine the fluid in the pericardial cavity, damage to the pericardium, damage to the heart valves, etc.
Specific Procedures

A lumbar puncture can help rule out infectious causes of neurological symptoms.
A biopsy (analysis of organ tissue) of the kidneys allows you to determine the type of glomerulonephritis and facilitate the choice of treatment tactics.
A skin biopsy allows you to clarify the diagnosis and exclude similar dermatological diseases.

Treatment of systemic lupus

Despite significant advances in the modern treatment of systemic lupus erythematosus, this task remains very difficult. Treatment aimed at eliminating the main cause of the disease has not been found, just as the cause itself has not been found. Thus, the principle of treatment is aimed at eliminating the mechanisms of the development of the disease, reducing provoking factors and preventing complications.

Eliminate physical and mental stress conditions
Reduce sun exposure, use sunscreen

Medical treatment

Glucocorticosteroids the most effective drugs in the treatment of SLE.

It has been proven that long-term glucocorticosteroid therapy in patients with SLE maintains a good quality of life and increases its duration.
Dosing regimens:

Inside:
- Initial dose of prednisolone 0.5 - 1 mg / kg
- Maintenance dose 5-10 mg
- Prednisolone should be taken in the morning, the dose is reduced by 5 mg every 2-3 weeks

High-dose intravenous methylprednisolone (pulse therapy)
- Dose 500-1000 mg/day, for 3-5 days
- Or 15-20 mg/kg body weight

This mode of prescribing the drug in the first few days significantly reduces the excessive activity of the immune system and relieves the manifestations of the disease.

Indications for pulse therapy: young age, fulminant lupus nephritis, high immunological activity, damage to the nervous system.

1000 mg methylprednisolone and 1000 mg cyclophosphamide on the first day

Cytostatics: cyclophosphamide (cyclophosphamide), azathioprine, methotrexate, are used in the complex treatment of SLE.

Indications:

Acute lupus nephritis
Vasculitis
Forms resistant to treatment with corticosteroids
The need to reduce doses of corticosteroids
High SLE activity
Progressive or fulminant course of SLE

Doses and routes of drug administration:

Cyclophosphamide with pulse therapy 1000 mg, then every day 200 mg until a total dose of 5000 mg is reached.
Azathioprine 2-2.5 mg/kg/day
Methotrexate 7.5-10 mg/week, by mouth

Anti-inflammatory drugs

They are used at high temperature, with damage to the joints, and serositis.

Naklofen, nimesil, aertal, catafast, etc.

Aminoquinoline drugs

They have an anti-inflammatory and immunosuppressive effect, are used for hypersensitivity to sunlight and skin lesions.

delagil, plaquenil, etc.

Biologicals are a promising treatment for SLE

These drugs have much fewer side effects than hormonal drugs. They have a narrowly targeted effect on the mechanisms of development of immune diseases. Effective but costly.

Anti CD 20 - Rituximab
Tumor necrosis factor alpha - Remicade, Gumira, Embrel

Other drugs

Anticoagulants (heparin, warfarin, etc.)
Antiplatelet agents (aspirin, clopidogrel, etc.)
Diuretics (furosemide, hydrochlorothiazide, etc.)
Calcium and potassium preparations

Methods of extracorporeal treatment

Plasmapheresis is a method of blood purification outside the body, in which part of the blood plasma is removed, and with it the antibodies that cause SLE disease.
Hemosorption is a method of purifying blood outside the body using specific sorbents (ion-exchange resins, activated carbon, etc.).

These methods are used in the case of severe SLE or in the absence of the effect of classical treatment.

What are the complications and prognosis for life with systemic lupus erythematosus?

The risk of developing complications of systemic lupus erythematosus directly depends on the course of the disease.

Variants of the course of systemic lupus erythematosus:

1. Acute course- is characterized by a lightning-fast onset, a rapid course and the rapid simultaneous development of symptoms of damage to many internal organs (lungs, heart, central nervous system, and so on). The acute course of systemic lupus erythematosus, fortunately, is rare, since this option quickly and almost always leads to complications and can cause the death of the patient.
2. Subacute course- characterized by a gradual onset, a change in periods of exacerbations and remissions, a predominance of general symptoms (weakness, weight loss, subfebrile temperature (up to 38 0

C) and others), damage to internal organs and complications occur gradually, not earlier than 2-4 years after the onset of the disease.
3. chronic course- the most favorable course of SLE, there is a gradual onset, damage mainly to the skin and joints, longer periods of remission, damage to internal organs and complications occur after decades.

Damage to organs such as the heart, kidneys, lungs, central nervous system, and blood, which are described as symptoms of the disease, in fact, are complications of systemic lupus erythematosus.

But it is possible to distinguish complications that lead to irreversible consequences and can lead to the death of the patient:

1. Systemic lupus erythematosus- affects the connective tissue of the skin, joints, kidneys, blood vessels and other body structures.

2. medicinal lupus erythematosus- unlike the systemic form of lupus erythematosus, a completely reversible process. Drug-induced lupus develops as a result of exposure to certain drugs:

Medicinal products for the treatment of cardiovascular diseases: phenothiazine groups (Apressin, Aminazine), Hydralazine, Inderal, Metoprolol, Bisoprolol, Propranolol and some others;
antiarrhythmic drug Novocainamide;
sulfonamides: Biseptol and others;
anti-tuberculosis drug Isoniazid;
oral contraceptives;
herbal preparations for the treatment of venous diseases (thrombophlebitis, varicose veins of the lower extremities, and so on): horse chestnut, venotonic Doppelhertz, Detralex and some others.

Clinical picture in drug-induced lupus erythematosus does not differ from systemic lupus erythematosus. All manifestations of lupus disappear after discontinuation of drugs , very rarely it is necessary to prescribe short courses of hormone therapy (Prednisolone). Diagnosis It is established by the method of exclusion: if the symptoms of lupus erythematosus began immediately after the start of taking medications and disappeared after their withdrawal, and reappeared after repeated use of these drugs, then we are talking about medicinal lupus erythematosus.

3. Discoid (or cutaneous) lupus erythematosus may precede the development of systemic lupus erythematosus. With this type of disease, the skin of the face is affected to a greater extent. Changes on the face are similar to those in systemic lupus erythematosus, but blood test parameters (biochemical and immunological) do not have changes characteristic of SLE, and this will be the main criterion for differential diagnosis with other types of lupus erythematosus. To clarify the diagnosis, it is necessary to conduct a histological examination of the skin, which will help to differentiate from diseases similar in appearance (eczema, psoriasis, skin form of sarcoidosis, and others).

4. neonatal lupus erythematosus occurs in newborn babies whose mothers suffer from systemic lupus erythematosus or other systemic autoimmune diseases. At the same time, the mother may not have symptoms of SLE, but autoimmune antibodies are detected during their examination.

Symptoms of neonatal lupus erythematosus the child usually manifests itself before the age of 3 months:

changes on the skin of the face (often look like a butterfly);
congenital arrhythmia, which is often determined by ultrasound of the fetus in the II-III trimesters of pregnancy;
lack of blood cells in the general blood test (decrease in the level of erythrocytes, hemoglobin, leukocytes, platelets);
detection of autoimmune antibodies specific for SLE.

All these manifestations of neonatal lupus erythematosus disappear after 3-6 months and without special treatment after maternal antibodies cease to circulate in the child's blood. But it is necessary to adhere to a certain regimen (avoid exposure to sunlight and other ultraviolet rays), with severe manifestations on the skin, it is possible to use 1% Hydrocortisone ointment.

5. Also, the term "lupus" is used for tuberculosis of the skin of the face - tuberculous lupus. Tuberculosis of the skin is very similar in appearance to the systemic lupus erythematosus butterfly. The diagnosis will help to establish a histological examination of the skin and microscopic and bacteriological examination of the scraping - Mycobacterium tuberculosis (acid-resistant bacteria) is detected.

Photo: this is what tuberculosis of the skin of the face or tuberculous lupus looks like.

Systemic lupus erythematosus and other systemic connective tissue diseases, how to differentiate?

Group of systemic connective tissue diseases:

Systemic lupus erythematosus.
Idiopathic dermatomyositis (polymyositis, Wagner's disease)- defeat by autoimmune antibodies of smooth and skeletal muscles.
Systemic scleroderma is a disease in which normal tissue is replaced by connective tissue (which does not carry functional properties), including blood vessels.
Diffuse fasciitis (eosinophilic)- damage to the fascia - structures that are cases for skeletal muscles, while in the blood of most patients there is an increased number of eosinophils (blood cells responsible for allergies).
Sjögren's syndrome- damage to various glands (lacrimal, salivary, sweat, and so on), for which this syndrome is also called dry.
Other systemic diseases.

Systemic lupus erythematosus has to be differentiated from systemic scleroderma and dermatomyositis, which are similar in their pathogenesis and clinical manifestations.

Differential diagnosis of systemic connective tissue diseases.

Diagnostic criteria	Systemic lupus erythematosus	Systemic scleroderma	Idiopathic dermatomyositis
The onset of the disease	weakness, fatigue; increase in body temperature; weight loss; violation of skin sensitivity; recurrent joint pain.	weakness, fatigue; increase in body temperature; violation of skin sensitivity, burning sensation of the skin and mucous membranes; numbness of the limbs; weight loss pain in the joints; Raynaud's syndrome - a sharp violation of blood circulation in the limbs, especially in the hands and feet. *Photo:* Raynaud's syndrome	severe weakness; increase in body temperature; muscle pain; there may be pain in the joints; stiffness of movements in the limbs; compaction of skeletal muscles, their increase in volume due to edema; swelling, cyanosis of the eyelids; Raynaud's syndrome.
Temperature	Prolonged fever, body temperature above 38-39 0 C.	Prolonged subfebrile condition (up to 38 0 C).	Moderate prolonged fever (up to 39 0 C).
Appearance of the patient (at the beginning of the disease and in some of its forms, the appearance of the patient may not be changed in all these diseases)	Skin lesions, mostly of the face, "butterfly" (redness, scales, scars). Rashes can be all over the body and on the mucous membranes. Dry skin, loss of hair, nails. Nails are deformed, striated nail plates. Also, throughout the body there may be hemorrhagic rashes (bruises and petechiae).	The face can acquire a “mask-like” expression without facial expressions, stretched, the skin is shiny, deep folds appear around the mouth, the skin is motionless, tightly soldered to deep-lying tissues. Often there is a violation of the glands (dry mucous membranes, as in Sjögren's syndrome). Hair and nails fall out. Dark spots on the skin of the extremities and neck against the background of "bronze skin".	A specific symptom is swelling of the eyelids, their color may be red or purple, on the face and in the décolleté area there is a varied rash with reddening of the skin, scales, hemorrhages, scars. With the progression of the disease, the face acquires a “mask-like appearance”, without facial expressions, stretched, may be skewed, and drooping of the upper eyelid (ptosis) is often detected.
The main symptoms during the period of disease activity	skin lesions; photosensitivity - skin sensitivity when exposed to sunlight (like burns); pain in the joints, stiffness of movements, impaired flexion and extension of the fingers; changes in the bones; nephritis (edema, protein in the urine, increased blood pressure, urinary retention and other symptoms); arrhythmias, angina pectoris, heart attack and other cardiac and vascular symptoms; shortness of breath, bloody sputum (pulmonary edema); intestinal motility and other symptoms; damage to the central nervous system.	skin changes; Raynaud's syndrome; pain and stiffness of movements in the joints; difficult extension and flexion of the fingers; dystrophic changes in the bones, visible on the x-ray (especially the phalanges of the fingers, jaw); muscle weakness (muscle atrophy); severe disorders of the intestinal tract (motility and absorption); violation of the heart rhythm (growth of scar tissue in the heart muscle); shortness of breath (overgrowth of connective tissue in the lungs and pleura) and other symptoms; damage to the peripheral nervous system.	skin changes; severe pain in the muscles, their weakness (sometimes the patient is unable to lift a small cup); Raynaud's syndrome; violation of movements, over time, the patient is completely immobilized; with damage to the respiratory muscles - shortness of breath, up to complete paralysis of the muscles and respiratory arrest; with damage to the masticatory muscles and muscles of the pharynx - a violation of the act of swallowing; with damage to the heart - rhythm disturbance, up to cardiac arrest; with damage to the smooth muscles of the intestine - its paresis; violation of the act of defecation, urination and many other manifestations.
Forecast	Chronic course, over time, more and more organs are affected. Without treatment, complications develop that threaten the life of the patient. With adequate and regular treatment, it is possible to achieve a long-term, stable remission.
Laboratory indicators	increase in gamma globulins; ESR acceleration; positive C-reactive protein; decrease in the level of immune cells of the complementary system (C3, C4); low amount of blood cells; the level of LE cells is significantly increased; positive ANA test; anti-DNA and detection of other autoimmune antibodies.		an increase in gamma globulins, as well as myoglobin, fibrinogen, ALT, AST, creatinine - due to the breakdown of muscle tissue; positive test for LE cells; rarely anti-DNA.
Principles of treatment	Long-term hormonal therapy (Prednisolone) + cytostatics + symptomatic therapy and other drugs (see article section "Treatment of systemic lupus").

As you can see, there is not a single analysis that would completely differentiate systemic lupus erythematosus from other systemic diseases, and the symptoms are very similar, especially in the early stages. Experienced rheumatologists often need to evaluate the skin manifestations of the disease to diagnose systemic lupus erythematosus (if present).

Systemic lupus erythematosus in children, what are the features of symptoms and treatment?

Systemic lupus erythematosus is less common in children than in adults. In childhood, rheumatoid arthritis is more often detected from autoimmune diseases. SLE predominantly (in 90% of cases) affects girls. Systemic lupus erythematosus can occur in infants and young children, although rarely, the largest number of cases of this disease occurs during puberty, namely at the age of 11-15 years.

Given the peculiarity of immunity, hormonal levels, growth intensity, systemic lupus erythematosus in children proceeds with its own characteristics.

Features of the course of systemic lupus erythematosus in childhood:

more severe disease , high activity of the autoimmune process;
chronic course disease in children occurs only in a third of cases;
more common acute or subacute course diseases with rapid damage to internal organs;
also isolated only in children acute or fulminant course SLE - almost simultaneous damage to all organs, including the central nervous system, which can lead to the death of a small patient in the first six months from the onset of the disease;
frequent development of complications and high mortality;
the most common complication is bleeding disorder in the form of internal bleeding, hemorrhagic eruptions (bruises, hemorrhages on the skin), as a result - the development of a shock state of DIC - disseminated intravascular coagulation;
systemic lupus erythematosus in children often occurs in the form of vasculitis - inflammation of the blood vessels, which determines the severity of the process;
children with SLE are usually malnourished , have a pronounced deficiency of body weight, up to cachexia (extreme degree of dystrophy).

The main symptoms of systemic lupus erythematosus in children:

1. The onset of the disease acute, with an increase in body temperature to high numbers (over 38-39 0 C), with pain in the joints and severe weakness, a sharp loss of body weight.
2. Skin changes in the form of a "butterfly" in children are relatively rare. But, given the development of a lack of blood platelets, a hemorrhagic rash is more common throughout the body (bruises for no reason, petechiae or pinpoint hemorrhages). Also, one of the characteristic signs of systemic diseases is hair loss, eyelashes, eyebrows, up to complete baldness. The skin becomes marbled, very sensitive to sunlight. There may be various rashes on the skin that are characteristic of allergic dermatitis. In some cases, Raynaud's syndrome develops - a violation of the circulation of the hands. In the oral cavity there may be long-term non-healing sores - stomatitis.
3. Joint pain- a typical syndrome of active systemic lupus erythematosus, the pain is periodic. Arthritis is accompanied by the accumulation of fluid in the joint cavity. Pain in the joints over time is combined with pain in the muscles and stiffness of movement, starting with the small joints of the fingers.
4. For children characterized by the formation of exudative pleurisy(fluid in the pleural cavity), pericarditis (fluid in the pericardium, the lining of the heart), ascites and other exudative reactions (dropsy).
5. Heart failure in children, it usually manifests as myocarditis (inflammation of the heart muscle).
6. Kidney damage or nephritis much more often develops in childhood than in adults. Such nephritis relatively quickly leads to the development of acute renal failure (requiring intensive care and hemodialysis).
7. Lung injury is rare in children.
8. In the early period of the disease in adolescents, in most cases, there is gastrointestinal tract injury(hepatitis, peritonitis, etc.).
9. Damage to the central nervous system in children it is characterized by capriciousness, irritability, in severe cases, convulsions may develop.

That is, in children, systemic lupus erythematosus is also characterized by a variety of symptoms. And many of these symptoms are masked under the guise of other pathologies, the diagnosis of systemic lupus erythematosus is not immediately assumed. Unfortunately, after all, timely treatment is the key to success in the transition of an active process into a period of stable remission.

Diagnostic principles systemic lupus erythematosus are the same as in adults, based mainly on immunological studies (detection of autoimmune antibodies).
In a general blood test, in all cases and from the very beginning of the disease, a decrease in the number of all blood cells (erythrocytes, leukocytes, platelets) is determined, blood clotting is impaired.

Treatment of systemic lupus erythematosus in children, as in adults, involves long-term use of glucocorticoids, namely Prednisolone, cytostatics and anti-inflammatory drugs. Systemic lupus erythematosus is a diagnosis that requires urgent hospitalization of the child in a hospital (rheumatology department, with the development of severe complications - in the intensive care unit or intensive care unit).
In a hospital, a complete examination of the patient is carried out and the necessary therapy is selected. Depending on the presence of complications, symptomatic and intensive therapy is carried out. Given the presence of bleeding disorders in such patients, injections of Heparin are often prescribed.
In the case of timely started and regular treatment, it is possible to achieve stable remission, while children grow and develop according to age, including normal puberty. In girls, a normal menstrual cycle is established and pregnancy is possible in the future. In this case forecast favorable for life.

Systemic lupus erythematosus and pregnancy, what are the risks and features of treatment?

As already mentioned, young women often suffer from systemic lupus erythematosus, and for any woman, the issue of motherhood is very important. But SLE and pregnancy is always a big risk for both the mother and the unborn baby.

Pregnancy risks for a woman with systemic lupus erythematosus:

1. Systemic lupus erythematosus In most cases does not affect the ability to get pregnant , as well as long-term use of prednisolone.
2. When taking cytostatics (Methotrexate, Cyclophosphamide and others), it is absolutely impossible to become pregnant , since these drugs will affect germ cells and embryonic cells; pregnancy is possible only not earlier than six months after the abolition of these drugs.
3. Half cases of pregnancy with SLE ends with the birth of healthy, full-term baby . At 25% cases such children are born premature , A in a quarter of cases observed miscarriage .
4. Possible complications of pregnancy in systemic lupus erythematosus, in most cases associated with damage to the vessels of the placenta:

fetal death;
. So, in a third of cases, an aggravation of the course of the disease develops. The risk of such deterioration is maximum in the first weeks of I, or in the III trimester of pregnancy. And in other cases, there is a temporary retreat of the disease, but for the most part, one should expect a strong exacerbation of systemic lupus erythematosus 1-3 months after birth. No one knows which path the autoimmune process will take.
6. Pregnancy can be a trigger in the development of the onset of systemic lupus erythematosus. Also, pregnancy can provoke the transition of discoid (cutaneous) lupus erythematosus to SLE.
7. Mother with systemic lupus erythematosus can pass genes to her baby that predispose him to develop a systemic autoimmune disease during his lifetime.
8. The child may develop neonatal lupus erythematosus associated with the circulation of maternal autoimmune antibodies in the blood of the baby; this condition is temporary and reversible.
- It is necessary to plan a pregnancy under the supervision of qualified doctors , namely a rheumatologist and a gynecologist.
- It is advisable to plan a pregnancy during a period of persistent remission chronic course of SLE.
- In case of acute systemic lupus erythematosus with the development of complications, pregnancy can adversely affect not only health, but also lead to the death of a woman.
- And if, nevertheless, pregnancy occurred during an exacerbation, then the question of its possible preservation is decided by the doctors, together with the patient. After all, exacerbation of SLE requires long-term use of drugs, some of which are absolutely contraindicated during pregnancy.
- Pregnancy is recommended no earlier than 6 months after discontinuation of cytotoxic drugs (Methotrexate and others).
- With lupus lesion of the kidneys and heart there can be no talk of pregnancy, this can lead to a woman's death from kidney and / or heart failure, because it is these organs that are under a huge load when carrying a baby.
Management of pregnancy in systemic lupus erythematosus:
1. Essential throughout pregnancy observed by a rheumatologist and an obstetrician-gynecologist , the approach to each patient is only individual.
2. Be sure to follow the rules: do not overwork, do not be nervous, eat normally.
3. Pay close attention to any changes in your health.
4. Delivery outside the maternity hospital is unacceptable , as there is a risk of developing severe complications during and after childbirth.
7. Even at the very beginning of pregnancy, a rheumatologist prescribes or corrects therapy. Prednisolone is the main drug for the treatment of SLE and is not contraindicated during pregnancy. The dose of the drug is selected individually.
8. Also recommended for pregnant women with SLE taking vitamins, potassium supplements, aspirin (up to the 35th week of pregnancy) and other symptomatic and anti-inflammatory drugs.
9. Mandatory treatment of late toxicosis and other pathological conditions of pregnancy in a maternity hospital.
10. After childbirth the rheumatologist increases the dose of hormones; in some cases, it is recommended to stop breastfeeding, as well as the appointment of cytostatics and other drugs for the treatment of SLE - pulse therapy, since it is the postpartum period that is dangerous for the development of severe exacerbations of the disease.
Previously, all women with systemic lupus erythematosus were advised not to become pregnant, and in the event of conception, all were recommended artificial termination of pregnancy (medical abortion). Now, doctors have changed their opinion on this matter, you can’t deprive a woman of motherhood, especially since there are considerable chances to give birth to a normal healthy baby. But everything must be done in order to minimize the risk to mother and baby.
Is lupus erythematosus contagious?
Of course, any person who sees strange rashes on the face thinks: “Maybe it’s contagious?”. Moreover, people with these rashes walk for so long, feel unwell and constantly take some kind of medication. Moreover, earlier doctors also assumed that systemic lupus erythematosus is transmitted sexually, by contact, or even by airborne droplets. But having studied the mechanism of the disease in more detail, scientists completely dispelled these myths, because this is an autoimmune process.
The exact cause of the development of systemic lupus erythematosus has not yet been established, there are only theories and assumptions. It all boils down to one thing, that the underlying cause is the presence of certain genes. But still, not all carriers of these genes suffer from systemic autoimmune diseases.
The trigger mechanism for the development of systemic lupus erythematosus can be:
- various viral infections;
- bacterial infections (especially beta-hemolytic streptococcus);
- stress factors;
- hormonal changes (pregnancy, adolescence);
- environmental factors (for example, ultraviolet radiation).
But infections are not causative agents of the disease, so systemic lupus erythematosus is absolutely not contagious to others.
Only tuberculous lupus can be contagious (tuberculosis of the skin of the face), since a large number of tuberculosis sticks are detected on the skin, while the contact route of transmission of the pathogen is isolated.

Lupus erythematosus, what diet is recommended and are there any methods of treatment with folk remedies?
As with any disease, nutrition plays an important role in lupus erythematosus. Moreover, with this disease, there is almost always a deficiency, or against the background of hormonal therapy - excess body weight, lack of vitamins, trace elements and biologically active substances.
The main characteristic of the SLE diet is a balanced and proper diet.
1. foods containing unsaturated fatty acids (Omega-3):
- sea fish;
- many nuts and seeds;
- vegetable oil in a small amount;
2. fruits and vegetables contain more vitamins and microelements, many of which contain natural antioxidants, the necessary calcium and folic acid are found in large quantities in green vegetables and herbs;
3. juices, fruit drinks;
4. lean poultry meat: chicken, turkey fillet;
5. low-fat dairy , especially dairy products (low-fat cheese, cottage cheese, yogurt);
6. cereals and vegetable fiber (grain bread, buckwheat, oatmeal, wheat germ and many others).
1. Foods with saturated fatty acids have a bad effect on blood vessels, which can aggravate the course of SLE:
- animal fats;
- fried food;
- fatty meats (red meat);
- dairy products with high fat content and so on.
2. Seeds and sprouts of alfalfa (bean culture).

Photo: alfalfa grass.
3. Garlic - powerfully stimulates the immune system.
4. Salty, spicy, smoked dishes holding fluid in the body.
If diseases of the gastrointestinal tract occur against the background of SLE or taking medications, then the patient is recommended frequent fractional meals according to a therapeutic diet - table number 1. All anti-inflammatory drugs are best taken with or immediately after meals.
Treatment of systemic lupus erythematosus at home is possible only after the selection of an individual treatment regimen in a hospital setting and the correction of conditions that threaten the life of the patient. Heavy drugs used in the treatment of SLE cannot be prescribed on their own, self-medication will not lead to anything good. Hormones, cytostatics, non-steroidal anti-inflammatory drugs and other drugs have their own characteristics and a bunch of adverse reactions, and the dose of these drugs is very individual. The therapy selected by doctors is taken at home, strictly adhering to the recommendations. Omissions and irregularity in taking medications are unacceptable.
Concerning traditional medicine recipes, then systemic lupus erythematosus does not tolerate experiments. None of these remedies will prevent the autoimmune process, you can just lose precious time. Folk remedies can give their effectiveness if they are used in combination with traditional methods of treatment, but only after consultation with a rheumatologist.
Some traditional medicines for the treatment of systemic lupus erythematosus:

Precautionary measures! All folk remedies containing poisonous herbs or substances should be out of the reach of children. One must be careful with such remedies, any poison is a medicine as long as it is used in small doses.
Photo, what do the symptoms of lupus erythematosus look like?

Photo: changes on the skin of the face in the form of a butterfly in SLE.

Photo: skin lesions of the palms with systemic lupus erythematosus. In addition to skin changes, this patient shows thickening of the joints of the phalanges of the fingers - signs of arthritis.

Dystrophic changes in nails with systemic lupus erythematosus: fragility, discoloration, longitudinal striation of the nail plate.

Lupus lesions of the oral mucosa . According to the clinical picture, they are very similar to infectious stomatitis, which do not heal for a long time.

And this is what they might look like early symptoms of discoid or cutaneous lupus erythematosus.

And this is what it might look like neonatal lupus erythematosus, these changes, fortunately, are reversible and in the future the baby will be absolutely healthy.

Skin changes in systemic lupus erythematosus characteristic of childhood. The rash is hemorrhagic in nature, reminiscent of measles rashes, leaves pigment spots that do not go away for a long time.

Photo of symptoms of lupus erythematosus

Lupus erythematosus in children is a disease that is directly related to the activity of the body's immune system. Doctors describe it as an autoimmune inflammation in which antibodies produced by the immune system destroy the body's DNA cells.

In addition, antibodies have the ability to destroy the connective tissues of internal organs, which causes damage and disruption (up to termination) of the functions of various organs and systems.

General picture of the disease

The disease is systemic in nature, so it cannot be completely cured. There are times when it progresses and requires urgent action, otherwise a fatal outcome is possible, and it happens that the coming weakening of the disease slowly drags on for years.

Lupus is more common in people between the ages of 20 and 40. It has long been proven that the female half of the population is doomed more than the male half. Blame the genetic characteristics of the female body.

Children are diagnosed with lupus erythematosus in 20% of all cases, the disease itself and the fight against it are much harder than in adults, often the fight ends in death. In very young children, lupus erythematosus is classified as an exception, but at the age of 9-10 years, its full manifestation is already possible.

This state of affairs is associated with the hormonal background of the body. It is at puberty, when there is an active release of hormones, that there is a possibility of the onset and development of the disease.

Causes of Lupus

Scientists put forward many theories, but how the disease lupus is not fully understood. However, most experts agree that the main cause of this autoimmune disorder is a viral infection.

In second place is the version about the effect of medications.: vaccines, antibiotics, anticonvulsants and antihypertensives, sulfonamides, gamma globulin.

The third place is given to factors that can increase the individual sensitivity of the organism.: hypothermia, excessive solar radiation, severe stress, etc.

The first signs of lupus erythematosus in children

It is almost impossible to immediately recognize lupus in a child. There are no classic symptoms. The disease begins with the defeat of one organ, then this so-called inflammation fades away, but symptoms of a completely different disease, characteristic of the next affected organ, appear.

Consider first warning signs:

Medical diagnosis of lupus erythematosus

The complete clinical picture of the disease is given only by additional laboratory tests. By the way, there are no separate tests specifically for lupus erythematosus. The doctor directs for diagnosis and analysis, based on the specific symptoms present.

Mandatory tests for lupus erythematosus:

general blood and urine tests;
blood chemistry;
blood test for antinuclear antibodies and antibodies against DNA cells.

Symptoms of lupus erythematosus and forms of this disease in children

The symptoms of lupus erythematosus depend on the form it takes. In medicine, there are three variants of the course of this disease.

Acute course

Has a progressive character. Symptoms:

small red rash on the bridge of the nose and cheeks;
sharp headaches;
general intoxication of the body;
severe fever;
aches in the joints;
marked decrease in overall activity.

During the first months, the kidneys are affected, resulting in the characteristic signs of kidney disease being added to the overall picture of symptoms.

Subacute course

In most cases, such lupus erythematosus begins with polyarthritis.. Those. conditions when several joints become inflamed at the same time or even alternately. Other symptoms:

red small rash on the face (mainly cheeks and bridge of the nose);
impaired renal function;
carditis (one of the disorders of the heart);
inflammation of the mucous membranes;
noticeable weight loss;
lack of appetite.

chronic course

Most difficult to diagnose. Lupus erythematosus first affects one organ. On the face there will be all signs of inflammation of this organ. Alternately, relapses of skin rashes or polyarthritis occur. Very slowly, the disease will involve other organs and systems in the process. This can go on for years.

Which doctor should I contact and what treatment can he offer?

The main treatment for lupus erythematosus is a rheumatologist. First of all, the doctor will prescribe a complex hormonal drug treatment. It is carried out in stationary conditions, which allows you to optimally select the doses of medicines and monitor. Antibiotics and multivitamins are recommended in cases where an infection is additionally attached to the disease.

Since the disease is systemic and affects many organs, treatment is carried out in parallel by a general practitioner, immunologist, neuropathologist, and endocrinologist.

After discharge, treatment is not stopped. At home, the patient must take corticosteroid drugs prescribed by the doctor at all times. A positive effect is expected only after a few months.

At the specified time, the patient is obliged to come for an examination to the doctor (after discharge - twice a month, a year later - twice a year).

What to remember after reading the article

Lupus erythematosus in children (just like in adults) is a systemic and incurable disease.
It manifests itself in a percentage ratio more in women / girls, since they often experience hormonal emissions and changes.
Causes of lupus: failure of the immune system when antibodies attack DNA cells, the influence of medications and external factors that increase the individual sensitivity of the body.
The first signs of lupus in children are very similar to the signs of any other disease, but adults should be most alerted by a rash on the bridge of the nose and cheeks in the form of a "butterfly".
There are three variants of the course of the disease: acute, subacute, chronic. Each carries its own symptoms.
It is quite difficult to immediately establish a diagnosis of "systemic lupus erythematosus" for a child. The disease is not fully understood. As already mentioned, there are no single characteristic signs, and also there are no special laboratory samples and analyzes.
When making a diagnosis, the doctor will proceed from the patient's individual symptoms and the results of his tests (based on the manifestation of all the same symptoms).

American scientists stepped forward in the treatment of lupus

Recently, American doctors have developed a new drug that should revolutionize the treatment of systemic lupus erythematosus (SLE): here. There you will also learn about the incubation period of meningitis.

If you doubt the baby's health, read about the symptoms of serous meningitis here: If you find obvious signs, contact your doctors immediately!

RCHD (Republican Center for Health Development of the Ministry of Health of the Republic of Kazakhstan)
Version: Clinical Protocols of the Ministry of Health of the Republic of Kazakhstan - 2015

Other forms of systemic lupus erythematosus (M32.8), Drug-induced systemic lupus erythematosus (M32.0), Systemic lupus erythematosus, unspecified (M32.9), Systemic lupus erythematosus affecting other organs or systems (M32.1+)

Pediatrics, Children's rheumatology

general information

Short description

Recommended
Expert Council
RSE on REM "Republican Center
health development"
Ministry of Health
and social development
Republic of Kazakhstan
dated November 6, 2015
Protocol #15

Protocol name: Systemic lupus erythematosus

Systemic lupus erythematosus(SLE) is a systemic autoimmune disease of unknown etiology, which is based on a genetically determined violation of immune regulation, which determines the formation of organ-specific antibodies to antigens of cell nuclei with the development of immune inflammation in the tissues of various organs.

SLE- this is one of the most severe diseases from the group of systemic diseases of the connective tissue, characterized by pronounced clinical polymorphism, chronic progressive course and, if untreated, a poor prognosis.

ICD-10 code(s):
M32 Systemic lupus erythematosus.
Excludes: lupus erythematosus (discoid) (NOS) (L93.0).
M32.0 Drug-induced systemic lupus erythematosus
M32.1 Systemic lupus erythematosus affecting other organs or systems.
M32.8 Other forms of systemic lupus erythematosus
M32.9 Systemic lupus erythematosus, unspecified

Abbreviations used in the protocol:

ACR-American College of Rheumatology, American College of Rheumatology
Αβ2-GP I -antibodies to beta2 glycoprotein
ALT -alanine aminotransferase
AZAazathioprine
ANA -antinuclear antibodies
Anti-Ro/SSA -antibodies to Ro/SSA antigen
Anti-Sm -antibodies to Sm antigen (Smith)
ACE -angiotensin converting enzyme
ASLO -antistreptolysin O
AST -aspartate aminotransferase
AFS -antiphospholipid syndrome
ACCP -antibodies to cyclic citrullinated peptide
APTT -activated partial thromboplastin time
ANCA-antibodies to neutrophil cytoplasm
ENA-antibodies to extractable nucleic antigen
BILAG-British Isles Lupus Assessment Group index, a specific index that evaluates the activity of SLE or the severity of an exacerbation in each individual organ or system
IVIGIntravenous immunoglobulin
TANK-blood biochemistry
GIBP -genetically engineered biological preparation
GIBT -genetically engineered biological therapy
GK -glucocorticoids
DNA -
GIT -Deoxyribonucleic acid
gastrointestinal tract
ELISA -linked immunosorbent assay
KFK -creatine phosphokinase
LE -lupus cells
LDH -lactate dehydrogenase
exercise therapy -physiotherapy
MMF -mycophenolate mofetil
MP -methylprednisolone
MTXmethotrexate
IPC -bone mineral density
NMG -low molecular weight heparin
ICD -international classification of diseases
INR -international normalized ratio
MRI -Magnetic resonance imaging
NSAIDs -non-steroidal anti-inflammatory drugs
UAC -general blood analysis
OAM -general urine analysis
PV -prothrombin time
Fri -pulse therapy
PTI -prothrombin index
PCR -polymerase chain reaction
RNA - antibodies to ribonucleic acid
RPGA -passive hemagglutination reaction
RIBT - treponema pallidum immobilization reaction
RIF -immunofluorescence reaction
RF -rheumatoid factor
MYSELF -macrophage activation syndrome
SELENA-SLEDAI-validated SLE activity index, modified during the study
SELENA SLICC/ACR-damage index developed by the International SLE Clinic Collaboration with the assistance of the American College of Rheumatology
SLE -systemic lupus erythematosus
GFR -glomerular filtration rate
SSD -systemic scleroderma
ESR -erythrocyte sedimentation rate
SRP -C-reactive protein
TV -thrombin time
TSH -thyroid-stimulating hormone
T3 -triiodothyronine
T4 -free thyroxine
TPO -thyroperoxidase
ultrasound -ultrasonography
ECG -electrocardiogram
Echo KG -echocardiogram
IgG, IgM, IgA -immunoglobulins G, M, A
UZDG -ultrasound dopplerography of blood vessels
HPN -chronic renal failure
CEC -circulating immunocomplexes
COX-2 -cyclooxygenase-2
CsA -cyclosporine A
CNS -central nervous system
FEGDS -fibroesophagogastroduodenoscopy
JIA -juvenile idiopathic arthritis
EEG -electroencephalography

Protocol development date: 2015

Protocol Users: pediatricians, rheumatologists, general practitioners, emergency physicians.

Evaluation of the degree of evidence of the given recommendations.
Evidence level scale:

A	High-quality meta-analysis, systematic review of RCTs, or large RCTs with a very low probability (++) of bias whose results can be generalized to an appropriate population.
IN	High-quality (++) systematic review of cohort or case-control studies or High-quality (++) cohort or case-control studies with very low risk of bias or RCTs with low (+) risk of bias, the results of which can be generalized to the appropriate population .
WITH	Cohort or case-control or controlled trial without randomization with low risk of bias (+). Results that can be generalized to an appropriate population or RCTs with very low or low risk of bias (++ or +) that cannot be directly generalized to an appropriate population.
D	Description of a case series or uncontrolled study or expert opinion.
GPP	Best Pharmaceutical Practice.

Classification

Clinical classification:
In accordance with the classification of V.A. Nasonova (1972,1986), establish the nature of the course, the degree of activity and the clinical and morphological characteristics of lesions of organs and systems.

Table 2.- Working classification of clinical variants of SLE (Nasonova V.A., 1979 - 1986)

Character currents disease	Phase and the degree of activity of the process	Clinical and morphological characteristics of lesions
Character currents disease	Phase and the degree of activity of the process	skin	joints	serous membranes	hearts	lungs	kidney	nervous system
Spicy subacute Chronic: - recurrent polyarthritis; - discoid lupus syndrome; - syndrome Raynaud; - Werlhof's syndrome; - syndrome Shegren	Phase; active Activity level: high (III); Moderate (II); Minimum (I); Phase; inactive (remission)	Symptom "butterflies" cappilla Rita Exudative erythema, purpura Discoid lupus, etc.	Arthralgia Acute, subacute and chronic polyarthritis	Polyserositis (pleurisy, effusion pericarditis, dry, adhesive perihepatitis, perisplenitis)	Myocarditis Endocarditis mitral valve insufficiency	Spicy, Chronic pneumonitis pneumosclerosis	Lupus Jade nephrotic, mixed iris urinary syndrome	Meningoencephalopyradiculoneuritis, polyneuritis

Degree of activity:
very high activity - IV (20 points and above);
high activity - III (11-19 points);
moderate activity - II (6-10 points);
minimal activity - I (1-5 points);
Lack of activity - 0 points.

Clinical manifestations:
Erythema;
Discoid lesions
Photosensitization;
The defeat of the mucous membranes;
non-erosive arthritis;
· Serositis;
· Damage to the kidneys;
damage to the nervous system;
Hematological disorders;
Immunological disorders;
· Positive antinuclear antibodies.

Lupus crises:
Monoorganic: renal, cerebral, hemolytic, cardiac, pulmonary, abdominal;
Multi-organ: renal-abdominal, renal-cardiac, cerebrocardial.

Clinical picture

Symptoms, course

Diagnostic Criteria for Making a Diagnosis[ 2- 7 ]:

Complaints and anamnesis:
In general, SLE in children is characterized by a more acute onset and course of the disease, earlier and more rapid generalization, and a less favorable outcome than in adults. . The debut of SLE can be either the defeat of one organ or system, or the involvement of several organs in the pathological process at once.

Complaints:
Increasing weakness, loss of appetite and body weight, intermittent or persistent fever;
Transient oligoarthritis or monoarthritis; migrating pain of varying intensity in large joints;
· Pain in the muscles;
Redness (erythema) of the skin of the cheeks and bridge of the nose - a symptom of a "butterfly", redness of the décolleté area, aggravated by excitement, exposure to the sun, exposure to frost and wind; polymorphic rashes on the skin;
Focal, diffuse, scarring and non-scarring alopecia, moderately painful ulcers on the mucous membranes of the lips, oral cavity, nasopharynx;
Pain in the heart, shortness of breath, palpitations, cough, chest pain, headache, convulsions (with the exception of metabolic, infectious and medicinal causes);
Pastosity or swelling of the eyelids, face;
recent weight loss;
· Various other complaints.

Anamnesis:
· Past viral infection, vaccination, prolonged exposure to the sun, swimming and sunbathing in water, severe emotional distress, allergies to medicines and food products;
Information about the presence in the family of relatives suffering from RH, allergies;
Presence of bad habits (smoking, alcohol);
Taking certain medications, hormonal drugs
Thrombosis in the child and in the mother.

Physical examination:
Depending on the affected organs or systems:
· Fever;
Rash on the skin of the cheeks: fixed erythema spreading to the bridge of the nose to the nasolabial zone.
· Discoid rash: erythematous raised plaques with adherent skin scales and follicular plugs, atrophic scarring may occur on older lesions.
· Skin rash resulting from a reaction to sunlight.
· Ulcers in the oral cavity: ulceration of the mouth, nose or nasopharynx, painless.
· Arthritis: non-erosive arthritis affecting one or two peripheral joints.
Serositis: pleurisy - pleural pain and / or pleural friction rub, pericarditis - pericardial rub during auscultation.
Renal damage: renal edema, arterial hypertension;
CNS damage: neuropsychic manifestations (convulsions, psychosis, etc.);
Damage to the digestive tract: nausea, vomiting, dysphagia, abdominal pain.
The diagnosis of SLE can be established with 95% specificity and 85% sensitivity if the patient has 4 out of 11 ACR criteria, 1997. If the patient has less than 4 diagnostic criteria, then the diagnosis of SLE is likely. If the ANA test is negative, then the patient has a very low probability of having SLE. Patients with an isolated positive ANA test without organ involvement or typical laboratory findings have a low probability of having SLE.

The nature of the flow:
. spicy- with a sudden onset, rapid generalization and the formation of a polysyndromic clinical picture, including damage to the kidneys and / or central nervous system, high immunological activity and often an unfavorable outcome in the absence of treatment;
. subacute- with a gradual onset, later generalization, undulation with the possible development of remissions and a more favorable prognosis;
. primary chronic- with a monosyndromic onset, late and clinically asymptomatic generalization and a relatively favorable prognosis.
Assessment of the degree of SLE activity is carried out to make a decision when choosing a treatment strategy according to the international SLE Activity Index SELENA / SLEDAI, in accordance with the severity of clinical symptoms and the level of laboratory parameters.
Signs of SLE in a patient during the 10 days preceding the examination are taken into account, regardless of their severity, improvement or deterioration (see Appendix). Interpretation of the value of the total score is carried out according to the classification of the degree of SLE activity (see paragraph 9 "Clinical classification", section III). The frequency of assessment of the degree of SLE activity is carried out at each visit of the patient. An increase in scores between two visits of 3-12 points is interpreted as a moderate exacerbation, more than 12 points - as a severe exacerbation of SLE.
Assessment of the degree of organ damage - cumulative damage to organs and systems associated with SLE itself, ongoing therapy, or the presence of concomitant diseases, is carried out using Damage Index SLICC/ACR. Determines the long-term prognosis of the disease and the appropriate treatment of damaged organs; is important for the medical and social examination. Irreversible tissue damage is organ damage in SLE that develops after the diagnosis of SLE and continues for more than 6 months. The frequency of the assessment is once a year (see Appendix). Signs that persist for more than 6 months are taken into account. The amount of points determines the degree of organ damage (see Table 3)

Table 3. Estimation of damage index (DI) of organs

Diagnostic criteria for secondary APS in SLE :
· Thrombosis - one or more episodes of arterial, venous or small vessel thrombosis in any organ.
Pathology of pregnancy - one or more cases of intrauterine death of a morphologically normal fetus after the 10th week of gestation, or one or more cases of preterm birth of a morphologically normal fetus before the 34th week of gestation, or three or more consecutive cases of spontaneous abortions before the 10th week of gestation gestation.

Laboratory criteria for API:
AT to cardiolipin (IgG and / or IgM) in the blood in medium or high titers in 2 or more studies with an interval of at least 12 weeks.
Plasma lupus anticoagulant in 2 or more studies at least 6 weeks apart.
AT to β2-GP I isotype IgG or IgM in medium or high titers in 2 or more studies with an interval of at least 12 weeks (standard ELISA).

Features of SLE in newborns(Oxford Handbook of Rheumatology - Handbook of Rheumatology, ed. A. Hakim, G. Clunie I. Haq, UK 2010):
Neonatal SLE is a rare condition characterized by discoid skin lesions, hemolytic anemia, hepatitis, thrombocytopenia, congenital heart block
Condition associated with transplacental transmission of maternal anti-Ro and anti-La antibodies
Non-cardiac manifestations resolve within the first year of life. Heart disease often requires early placement of an artificial pacemaker, and mortality in the first 3 years of life reaches 30%.
Women with anti-Ro and anti-La antibodies have a 5% chance of having their first child with congenital heart block, with subsequent pregnancies increasing the risk to 15%
· In the antenatal period, it is important to monitor the fetus, including DEHOKG.

Diagnostics

Diagnostic studies:

The main (mandatory) diagnostic examinations carried out at the outpatient level:
· Complete blood count 6 parameters on the analyzer;
· Biochemical blood test (determination of CRP, ASLO, RF, glucose, total protein, urea, creatinine, ALT, ACT);
· Determination of ANA, antibodies to double-stranded DNA;

· OAM;
EKG.

Additional diagnostic examinations performed at the outpatient level:
· Biochemical analysis of blood (determination of protein fractions, cholesterol, lipid fractions, CPK, LDH potassium, sodium, calcium, chlorides, alkaline phosphatase);

· Determination of RF, ACCP by ELISA;

ELISA (determination of antigen and antibodies to hepatitis B and C viruses);
ELISA (determination of total antibodies to HIV);
Bacteriological examination of discharge from the pharynx and nose (isolation of pure culture) with the determination of sensitivity to antibiotics;
Microscopy for Mycobacterium tuberculosis of urine and sputum 3 times (with specific radiological changes);
ECHOCG;
Ultrasound of the abdominal organs and kidneys;
X-ray of the chest;
X-ray densitometry of the spine and proximal femur (central or axial DEXA densitometry);
Tuberculin test - Mantoux test.

The minimum list of examinations that must be carried out upon referral for planned hospitalization: in accordance with the internal regulations of the hospital, taking into account the current order of the authorized body in the field of healthcare.

Basic (mandatory) diagnostic examinations carried out at the hospital level(in case of emergency hospitalization):
· Complete blood count of 6 parameters on the analyzer (at least 10 days);
Biochemical blood test (determination of CRP, ASLO, RF, glucose, total protein, urea, creatinine, ALT, ACT) (at least 10 days);
· Research of the general analysis of urine on the analyzer (physical and chemical properties with calculation of quantity of cellular elements of an uric sediment);
Determination of daily proteinuria;
Coagulogram: APTT, PV, PTI, INR, TV, RMFC, fibrinogen;
· Determination of antinuclear autoantibodies (ANA), antibodies to double-stranded DNA;
· Determination of lupus anticoagulant (LA1/LA2) in blood plasma;
· Determination of antibodies to cardiolipin in blood serum by ELISA method;
Immunological blood test (immunogram, CEC, immunoglobulins A, M, G, complement components (C3, C4);
· Microprecipitation reaction with serum cardiolipin antigen;
Ultrasound examination of one joint of the limbs;
X-ray survey of the chest (1 projection);
ECG;
echocardiography;
Ultrasound of the abdominal organs and kidneys.

Additional diagnostic examinations carried out at the hospital level:
· Biochemical analysis of blood (determination of CPK, LDH, serum iron, ferritin, amylase, triglycerides, high and low density lipoproteins, alkaline phosphatase) in the blood serum on the analyzer;
LE cells;
Determination of glomerular filtration according to Schwartz;
· Electrophoresis of protein fractions in blood serum and other biological fluids on the analyzer;
Determination of anti-TPO, antibodies to TG, TSH, T4, T3 in blood serum by ELISA method (according to indications);
Determination of cortisol in blood serum by ELISA method (according to indications);
Determination of HBsAg in blood serum by ELISA method (confirmatory);
Determination of total antibodies to hepatitis C virus in blood serum by ELISA method (according to indications);
· Determination of Ig G, Ig M to herpes simplex viruses type 1 and 2 (HSV-I, II), to the nuclear antigen of the Epstein Barr virus (HSV-IV), to cytomegalovirus (CMV-V) in blood serum by ELISA method;
Bacteriological examination of blood for sterility on the analyzer (according to indications);
· Bacteriological examination of discharge from the pharynx, wounds, eyes, ears, urine, bile, etc. by manual method (isolation of pure culture);
· Determination of sensitivity to antimicrobial preparations of isolated cultures by manual method;
· Detection of occult blood in the feces (hemocult test) by express method (according to indications);
· Urine culture tank;
· Bacteriological examination of transudate, exudate for sterility on the analyzer (according to indications);
· Sternal puncture - diagnostics (according to indications);
· Kidney biopsy with examination of biopsy light, imm-fluor, electr. microscopy;
· Calculation of myelogram and characterization of bone marrow hematopoiesis by manual method (for all patients - in a serious condition that does not correspond to the severity of SLE);
Trepanobiopsy - diagnostics (in the presence of destruction of the joints and bones of the skeleton, atypical for SLE);
Open biopsy of the lymph node (with severe lymphadenopathy or atypical severe general condition);
Fibroesophagogastroduodenoscopy;
Ultrasound of the thyroid gland (according to indications);
· Holter monitoring of the electrocardiogram (24 hours) (in violation of the rhythm and conduction of the heart);
Ultrasound of the vessels of the upper and lower extremities (according to indications);
Computed tomography of the chest and mediastinum (if a malignant neoplasm is suspected);
Computed tomography of the abdominal cavity and retroperitoneal space with contrast (if a malignant neoplasm is suspected);
Magnetic resonance imaging of the brain (on the recommendation of a neurologist);
· Magnetic resonance imaging of the abdominal cavity and retroperitoneal space with contrast (if a malignant neoplasm is suspected);
X-ray of the joints (according to indications);
X-ray densitometry of the lumbar spine (for patients receiving glucocorticoid therapy);
Electroencelography (in case of damage to the central nervous system);
Needle electromyography (according to indications);
Ophthalmoscopy (on the recommendation of an ophthalmologist);
· Consultation of specialists (according to indications).

Diagnostic measures taken at the stage of emergency care:
UAC;
EKG.

Instrumental examination:
· Chest X-ray- signs of infiltrates, pleurisy (exudative and dry), more often bilateral, less often signs of pneumonitis. Rarely, signs of pulmonary hypertension, usually as a consequence of recurrent pulmonary embolism in APS. Also, in order to exclude tuberculosis when prescribing GIBT
· Electrocardiography - assessment of cardiac activity;
· Ultrasound of the abdominal organs and kidneys - determination of the state of the abdominal organs, diagnosis of visceritis;
· Echocardiography of the heart- signs of pericarditis, myocarditis and endocarditis, as well as signs of pulmonary hypertension.
· X-ray of joints, bones - epiphyseal osteoporosis, mainly in the joints of the hands, less often in the carpometacarpal and radiocarpal joints, thinning of the subchondral plates, small usura of the articular bones (only in 1-5% of cases) with subluxations;
· Radiography of the pelvic bones- detection of aseptic necrosis of the femoral head.
· Ultrasound of the joints - possible presence of effusion and thickening of the synovial membrane of the joints
· Esophagogastroduodenoscopy- damage to the esophagus is manifested by its dilatation, erosive and ulcerative changes in the mucous membrane; often found ulceration of the mucous membrane of the stomach and duodenum.
· High resolution computed tomography- signs of pleurisy with or without effusion, interstitial pneumonia, diaphragmatic myopathy (myositis), basal discoid (subsegmental) atelectasis, acute lupus pneumonitis (based on pulmonary vasculitis) (according to indications)
· Kidney biopsy- according to the results of a kidney biopsy, it is possible to establish the severity and activity of the kidney damage, the involvement of the vessels and the tubular apparatus of the kidney; alternative causes of renal failure (eg, drug tubular) may also be identified.
· Dual Energy X-Ray Absorptiometry (DXA) - in osteoporosis, the level of the BMD T-criterion is ≤-2.5 SD.

Indications for consultation of narrow specialists:
Consultation with a nephrologist - in order to determine the tactics of treatment for LN;
consultation of a neurologist - with the development of neurological symptoms; as well as with the development of PML, in patients on immunosuppressive therapy, including rituximab;
consultation of a psychiatrist - in the presence of psychotic disorders to resolve the issue of prescribing psychotropic therapy, the need for treatment in a specialized hospital (psychosis, depression, accompanied by suicidal thoughts);
consultation of an ophthalmologist - in case of visual disturbances;
consultation of an obstetrician-gynecologist - according to indications;
Consultation of a surgeon - in the presence of abdominal pain with vomiting "coffee grounds" and diarrhea;
consultation of an angiosurgeon - in case of APS with vascular thrombosis;
consultation of an endocrinologist - with autoimmune thyroiditis and other endocrine pathology;
consultation of an infectious disease specialist - in case of suspicion of the development of an intercurrent infection;
consultation of a hematologist, oncologist - in case of suspected oncohematological disease
consultation of a gastroenterologist - with damage to the oral mucosa, with dysphagia (often associated with Raynaud's phenomenon), with anorexia, nausea, vomiting, diarrhea, peptic ulcers.

Laboratory diagnostics

Laboratory examination [2 - 4, 6,10]:

Non-specific:
· UAC: increased ESR, leukopenia (usually lymphopenia), thrombocytopenia;
it is possible to develop autoimmune hemolytic anemia, hypochromic anemia associated with chronic inflammation, latent gastric bleeding, or taking certain drugs.
· OAM: proteinuria, hematuria, leukocyturia, cylindruria.
· TANK: with predominant damage to internal organs at various periods of the disease: liver, pancreas.
· Coagulogram, determination of adhesion and platelet aggregation functions: control of hemostasis, thrombosis markers in APS, control of the platelet link of hemostasis;

Specific:
Immunological studies:
· ANA- a heterogeneous group of antibodies that react with various components of the nucleus. The sensitivity of this test is very significant (detected in 95% of patients with SLE), but the specificity is low. Often, ANA is detected in patients with other rheumatic and non-rheumatic diseases.
· Anti-dsDNA- is essential for assessing the activity of the disease, predicting the development of exacerbations and the effectiveness of the therapy. The anti-dsDNA test may be negative early in the course of the disease, after treatment, or during a period of clinical remission. A negative result in any period of the disease does not exclude SLE (detected in 20-70% of patients with SLE).
· A antiphospholipid antibodies(AT to cardiolipin, AT to b2-glycoprotein 1, lupus anticoagulant) is detected in 35-60% of children with SLE and are markers of antiphospholipid syndrome.
· Decreased total hemolytic activity of complement(CH50) and its components (C3 and C4) usually correlates with the activity of lupus nephritis, in some cases it may be the result of a genetically determined deficiency.
· Rheumatoid factor- autoantibodies of the IgM class, reacting with the Fc fragment of IgG, are often detected in SLE patients with severe articular syndrome.
· LE- cells- polymorphonuclear neutrophils (rarely eosinophils or basophils) with a phagocytosed cell nucleus or its individual fragments - are formed in the presence of antibodies to the DNA-histone complex and are detected on average in 60-70% of children with SLE.

Differential Diagnosis

Differential Diagnosis

Table 4 - Differential diagnosis.

Disease	Difference with SLE
JIA	Joint damage is persistent, progressive. Great morning stiffness. As the disease progresses, destruction of the articular surfaces and deformity of the joints develop. Typical erosive changes on the R-mm. Severe damage to internal organs.
Juvenile dermatomyositis	Characteristic skin manifestations (paraorbital lilac erythema, Gottron's syndrome, erythema over the elbow and knee joints), progressive muscle weakness, increased transaminases, CPK, aldolase.
Systemic vasculitis	Clinical symptoms are determined by ischemic changes in organs and tissues due to inflammation and necrosis of the vascular wall. The defeat of the nervous system mainly in the form of multiple mononeuritis. Leukocytosis, thrombocytosis, ANCA positive
juvenile scleroderma	Typical changes in the skin and subcutaneous fat, damage to the gastrointestinal tract. X-ray signs (osteolysis, resorption of terminal phalanges), soft tissue calcification.
Idiopathic thrombocytopenic purpura	Hemorrhages on the skin and mucous membranes (from petechiae to large ecchymosis). Bleeding from the mucous membranes of the nose, gums, etc. Positive endothelial tests Thrombocytopenia Increase bleeding time Decreased retraction of the blood clot
Viral arthritis	epidemiological history. Spontaneous regression of clinical symptoms.
drug lupus syndrome	Long-term use of drugs that can induce lupus-like syndrome (antihypertensive, antiarrhythmic, anticonvulsant drugs, perioral contraceptives). Severe kidney damage, central nervous system, thrombocytopenia are rare. After discontinuation of the drug, clinical symptoms regress within 4-6 weeks. (positive ANA test lasts up to 1 year)
Malignant neoplasms	Results of oncological search.

It is extremely important to distinguish an exacerbation of SLE from an acute infectious disease (infective endocarditis, tuberculosis, yersiniosis, Lyme disease, etc.).

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Treatment

Purpose of treatment :
Decreased disease activity
prevention of irreversible damage and death;
Improving the quality of life and social adaptation (D);
Reducing the risk of side effects, especially when prescribing HA and CT (C);
· The goals of treatment should be agreed with the patient, depending on the individual manifestations of SLE (D);

Basic principles of SLE treatment in children:
individual approach in choosing the most rational treatment regimen, taking into account the clinical manifestations, the degree of activity and the nature of the course of the disease, as well as the constitutional features and response of the child's body to treatment;
complexity;
Programming (correct and consistent implementation of all components of the therapeutic program chosen for treatment);
continuity (timely alternation of intensive immunosuppressive and maintenance therapy, taking into account the phase of the disease);
Constant monitoring of the effectiveness and safety of the therapy;
Duration and continuity
phasing.

Treatment tactics:
With the subsidence of activity and the development of remission, outpatient treatment is recommended;
In the acute period of SLE, the issue of inpatient treatment is decided.

Non-drug treatment:
Decreased psycho-emotional load;
Reducing sun exposure, active treatment of concomitant diseases;
avoid the introduction of vaccines and therapeutic sera;
In order to prevent osteoporosis, smoking cessation is recommended (recommendation grade D), eating foods high in calcium, potassium, and exercising;
low-fat and low-cholesterol diet, weight control and exercise are indicated (recommendation grade D);
Consider the risk of thrombosis and the need for anticoagulant therapy.

Medical treatment:
Therapy for SLE is based on pathogenetic principles and is aimed at suppressing the synthesis of autoantibodies, reducing the activity of immune inflammation, and correcting hemostasis;
Determined for each child individually, taking into account his constitutional features, clinical manifestations and SLE activity;
the effectiveness of previous therapy and its tolerability, as well as other parameters;
treatment is carried out for a long time and continuously;
timely alternate intensive and maintenance immunosuppressive therapy, taking into account the phase of the disease;
To constantly monitor its effectiveness and safety.

Basic drugs(table 3 and 4):
Hormonal immunosuppressants: glucocorticoids (metipred, prednisolone, 6-MP) are first-line drugs for SLE. They have anti-inflammatory, immunomodulatory and anti-destructive effects. They inhibit all phases of inflammation, proliferation of lymphoid tissue, reduce the cytotoxic activity of T-lymphocytes, the concentration of immunoglobulins (level of evidence A). Side effects: hyperglycemia, osteoporosis, hypertension, glaucoma, gastrointestinal manifestations, myopathy, progressive atherosclerosis.
Aminoquinoline derivatives ( hydroxychloroquine sulfate, chloroquine diphosphate ) prevent the development of exacerbations of SLE, lower lipid levels and reduce the risk of developing visceral lesions, thrombotic complications, the risk of developing cardiovascular complications and contributes to increased survival. Aminoquinoline drugs in the absence of contraindications should be prescribed to all patients with SLE without exception (Evidence level A). Side effect: central scotoma at any stage of the disease.
Additional drugs (table 3 and 4):
Non-hormonal immunosuppressants, cytostatics(CF, AZA, MTX, MMF, Cs A) have an anti-inflammatory effect, the ability to suppress the immune complex anti-inflammatory process and autoantibody formation. Cytostatics are an essential component of the treatment of SLE, especially in lupus crises, a threatening course with damage to the kidneys, central nervous system, generalized vasculitis, alveolitis. The appointment of cytostatics, both in the induction phase and during maintenance therapy, should be under constant control. Side effects: severe infections, alopecia, suppression of bone marrow function, malignant neoplasms, infertility, hepatitis, nephrotoxicity, etc.
Non-steroidal anti-inflammatory drugs (NSAIDs)(ibuprofen, diclofenac, nimesulide) have a pronounced anti-inflammatory effect, a moderate immunosuppressive effect, and stabilize lysosomal membranes. In standard therapeutic doses, it can be used to treat the musculoskeletal manifestations of SLE, fever and moderate serositis. In secondary APS, it should be used with caution, because. may contribute to the development of thrombosis.
Immunotherapy ( intravenous immunoglobulin, rituximab ).
Intravenous immunoglobulin is indicated to increase the content of antibodies in the blood to a physiological level, to prevent infectious diseases and create passive immunity. It contributes to a more rapid decrease in the activity of the disease, reduces the risk of possible side and infectious complications, and allows to reduce the dose of prednisolone.
Rituximab (mabthera)- a genetically engineered biological drug inhibits B-cell proliferation - increases the efficiency and reduces the risk of complications in the treatment of SLE.
It is recommended to prescribe SLE patients with high immunological and clinical activity (high level of anti-DNA, decrease in C3 and C4 complement components, SLEDAI 6-10 points.
The appointment of GIBP is possible in case of proven (at the meeting of the CWC) ineffectiveness of standard therapy and with the written consent of the legal representatives of the child.
Patients must be informed about the need for early recognition of symptoms of infectious complications, and if appropriate signs appear (chills, fever, symptoms of urinary tract infection, upper respiratory tract infection, hepatitis, herpes, neurological disorders), immediately consult a doctor. Instructions for medical use should always be with the patient when prescribing Rituximab.
Ensure continuity in the appointment of the GIBT by:
maintaining a database of SLE patients selected for GIBT;
issuance to a patient receiving GIBT of a discharge summary to the clinic at the place of attachment;
informing PHC about all cases of refusal/cancellation of GIBT.
· Monitoring the effectiveness of GIBT is carried out by a rheumatologist every 1-3 months of treatment. When the goal of therapy is achieved, monitoring can be carried out less often - every 6-12 months.
· Patients who are on GIBT should be under dispensary observation at the place of residence.
· Patients who systematically violate the regimen of GIBT and the preventive recommendations of the attending physician are excluded from receiving the guaranteed volume of hospital-replacing medical care by decision of the Commission.

In addition, in the treatment of SLE, according to indications, the following are used:
- anticoagulant, antiplatelet, antihypertensive JIC, hepato-gastroprotectors, diuretics, antibiotics, folic acid, drugs for the prevention and treatment of osteoporosis and other symptomatic drugs.

Medical treatment provided on an outpatient basis:

Table 5 - Basic and additional drugs:

INN	Therapeutic range	A course of treatment
Used in combination.
Methylprednisolone (UD - A)
Hydroxychloroquine sulfate (UD - A)
Mycophenolate mofetil (UD - D)
100% cast chance): monotherapy, one of the following drugs is recommended
	From 6 years old and adolescents are prescribed at the rate of 0.5-2 mg / kg of body weight per day, divided into 2-3 doses	Average 4-6 weeks
ibuprofen	Assign a dose of 5 - 10 mg / kg / day in 3 - 4 doses. The maximum daily dose is 20 mg/kg.	On average 4-6 weeks.
Nimesulide (Nimesil)	3-5 mg / kg of body weight 2-3 times / day, the maximum dose is 5 mg / kg / day in 2-3 doses. Adolescents weighing over 40 kg are prescribed 100 mg 2 times / day.	Average 2 weeks
Naproxen	from 1 to 5 years - 2.5-10 mg / kg of body weight in 1-3 doses, children over 5 years old - 10 mg / kg per day in 2 doses	2 weeks
Mycophenolate mofetil (UD - D)	400 - 600 mg / m2 2 times a day with an interval of 12 hours, (no more than 2 g)	9 months and more against the background of combination therapy with GC, a maintenance dose of 1 g / day.
Cyclophosphamide (UD - A)	Low doses 500mg IV over an hour; High doses 0.5 mg - 1.0 g/m2 IV	Every 2 weeks, a total of 6 infusions, in combination with GCS, then 1 time in 3 months. up to 2 years, followed by maintenance therapy with MMF or AZA Monthly, 6 infusions in combination with corticosteroids
Azathioprine (UD - C)
Methotrexate (UD - A)	5-10.0 mg/m2 body surface per week orally or IM	Within 6 months and more
Cyclosporin A

Medical treatment provided at the inpatient level:

Table 6 - Basic and additional drugs:

INN	Therapeutic range	A course of treatment
Essential drugs (100% chance of use): are used in combination with methylprednzolone and hydroxychloroquine sulfate.
Methylprednisolone (UD - A)	0.5-1.0-1.5 mg/kg orally (2/3 DM in the morning)	Overwhelming dose of 4-6 weeks (no more than 8), the maintenance dose should not be less than 10-15 mg / day. (less than 0.2 mg/kg/day)
Hydroxychloroquine sulfate (Plaquenil) (UD-A)	0.1 -0.4 g / day (up to 5 mg / kg per day)	Within 2-4 months. then the dose is reduced by 2 times and it is recommended to use the drug for a long time (1-2 years or more).
Pulse therapy 6 MP (UD - A)	250 - 1000 mg / day. (no more)	in / in for 45 minutes, 3 days in a row, according to indications - repeat after 10 - 14 days
Additional medicines (less than100% cast chance). Monotherapy is recommended: one of the following drugs.
Mycophenolate mofetil (UD - D)	400 - 600 mg / m2 2 times a day with an interval of 12 hours, (no more than 2 g)		9 months and more against the background of combination therapy with GC, a maintenance dose of 1 g / day.
Azathioprine (UD - C)	1.0-3.0 mg / kg per day (the number of leukocytes in the blood should not be lower than 4.5-5.0 x 109 / l)		Duration of admission - at least 2 years against the background of combination therapy with GC
Diclofenac (Voltaren, Ortofen)	From 6 years old and adolescents are prescribed at the rate of 1-2 mg / kg of body weight per day, divided into 2-3 doses.
ibuprofen	Assign at a dose of 5 - 10 mg / kg / day in 3 - 4 doses, the maximum daily dose of 20 mg / kg.		On average 4-6 weeks, until a therapeutic effect is achieved
Methotrexate, oral, Methoject, IM (LE - A)	7.5-10.0 mg/m2 body surface per week orally or IM		For 6 months or more against the background of combination therapy with GC
Cyclosporin A	from 2.0-2.5 mg / kg per day orally and more, taking into account the tolerability of the drug, but less than 5 mg / kg per day.		18-24 months or more against the background of combination therapy with GC
Normal human immunoglobulin (UD - C)	1.0-2.0 g/kg per course; 0.4-0.5 g/kg for the treatment of infections		3 - 5 days
Rituximab	at a dose of 375 mg/m2 once a week		Within 18 months. and more
Pentoxifylline	Intravenous drip at a dose of 20 mg per year of life per day, the administration of the drug is divided into 2 doses		Within 12-14 days, then it is necessary to switch to oral administration of the drug in the same dose. The duration of treatment is 1-3 months. And more, against the background of the main therapy
Low molecular weight heparins: 1. Heparin	1. 200 - 400 IU / kg per day or more (prolonging the blood clotting time by 2 times), injected subcutaneously every 6-8 hours.		1. The duration of heparin therapy is 4-8 weeks. (in the absence of effect, therapy is continued with indirect anticoagulants)
Indirect anticoagulants (warfarin)	2.5-10 mg 1 time per day at the same time. The initial dose for patients who have not previously used warfarin is 5 mg per day (2 tablets) for the first 4 days. On the 5th day of treatment, MHO is determined.		The maintenance dose of the drug should keep the INR at the level of 2.0-3.0.
Antihypertensive JICs: ACE inhibitors: 1. Captopril (capoten) 2. Enalapril 3. Fosinopril ARBs (angiotensin receptor blockers): 1. Losartan β-blockers: 1. Atenolol : 1. Nifedipine (Corinfar)	ACE inhibitors: 1. 0.3-1.5 mg/kg/day, 2.0.1-0.6 mg / kg / day, 3. 5-10 mg/day. BRA: 1. 0.7-1.4 mg/kg/day, maximum 100 mg/day, from 6 years (D) β-blockers: 1. 1-2 mg/kg, maximum dose 100 mg/day. Calcium channel blockers : 1. 0.5-2 mg / kg / day in 2-3 doses.
Diuretics; 1. Furosemide 2. Spirinolactone 3. Combination of diuretics and albumin 20%	1. 4 - 6 mg / kg / day intravenously 3-4 times a day at regular intervals. 2. 2 - 4 mg/kg 3-4 times a day 3. 20% albumin 1g/kg 2-4 hours + furosemide 1-2mg/kg IV)		These drugs are used as monotherapy, in the absence of effect in combination until a therapeutic effect is achieved.
Concomitant Therapy: 1. Antibiotics; 2. Antifungal; 3. Hepatoprotectors; 4. Gastroprotectors; 5. Anti-osteoporotic; 6. Iron preparations; 7. Folic acid (except on the day of taking MTX); 8. Statins; 9. Neuroprotectors; 10. Fresh frozen plasma; 11. Dextrans.	Doses of drugs are selected based on kg / body weight of children, antibiotics by sensitivity		According to indications, until a therapeutic effect is achieved

Table 7 - Differentiated therapy for SLE (Recommendations of the APP, 2012):

SLE options	Treatment standards
Serositis:	Medium doses of oral HA (25-40 mg/day) or pulse therapy, Plaquenil 200-400 mg/day or Azathioprine 100-150 mg/day are used to maintain the effect and reduce the dose of HA (C)
With recurrent or life-threatening serositis	Used MMF (2 g/day), Cyclophosphamide (up to 3-4 g in total) or Rituximab 1000-2000 mg per course (C)
Lupus arthritis:	Medium and low dose GCs, azathioprine, plaquenil, and methorexate (C) in the absence of a lasting effect: MMF, Cyclosporine. Rituximab (C)
Neuropsychiatric manifestations: convulsive syndrome, transverse myelitis, psychosis, optic nerve damage, cerebrovasculitis	Immediate: high doses of HA (0.5–1.0 mg/kg), 6-MP pulse therapy, and cyclophosphamide infusions (500–1000 mg) are given (A) With insufficient efficiency and a life-threatening condition, the following is prescribed: - Rituximab (infusion 500 mg x 4); - IVIG (0.5-1.0 g/kg 3-5 days) - Plasmapheresis/immunosorption (C)
Hemolytic anemia, thrombocytopenia, leukopenia:	HA 0.5 to 1.0 mg/kg daily + azathioprine 100-200 mg daily (C) Cyclophosphamide (500-1000 mg infusions), IVIG (0.5 mg/kg for 1-3 days), Rituximab (500 mg x 4 or 1000 mg infusions 1-2 times) may be used if there is insufficient effect and specific antibodies are detected (C) If ineffective in individual patients: immunosorption, MMF, Cyclosporine, Splenectomy (C) Standard of care for ITTP: oral high-dose HA, Pulse therapy, Immunosorption, Plasmapheresis, CF, or Rituximab
Lupus pneumonitis: Hemorrhagic alveolitis: Interstitial pneumonitis, chronic course:	HA 0.5 to 1.0 mg/kg daily + Cyclophosphamide 500-1000 mg infusion monthly (C) Immediate Pulse therapy 6-MP + Cyclophosphamide (500–1000 mg infusions), Plasmapheresis, IVIG (0.5 mg/kg 1–3 days), Rituximab (500 mg x 4 or 1000 mg infusions 1–2 times) (C) monthly infusions of Cyclophosphamide 500-1000 mg + 6-MP 500-1000 mg If not effective: Rituximab 500-1000 mg every 3-6 months
Lupus nephritis according to the morphological type of nephritis:	If class I or II is detected, suppressive immunosuppressive and GC therapy is not prescribed (C) In the presence of class III or IV, therapy with massive doses of HA and CF (A) or MMF (B) is prescribed In cases of combination V with III \ IV classes, the same therapy is carried out as in IV (B) Class V - "pure mebranous VL" - large doses of HA and MMF are prescribed (C)
Induction therapy for LN class III/IV	1. Pulse therapy 6-MP (3 days, 500-1000 mg, no more) IT Options - MMF 2-3 g/day min. - 6 months "high doses" - ZF infusion 0.5g - 1g + 6-MP 0.5g - 1g - 6 months. "low doses" - ZF 500 mg 1 time in 2 weeks - 6 doses 2. Glucocorticoids - 0.5-1.0 mg / kg 3. INEFFICIENCY RITUXIMAB
VN with IV or IV \ V class with the presence of crescents	Pulse therapy with 6 methylprednisolone and oral GCs at doses of at least 1 mg/kg/day. "high" or "low" doses of CF or MMF 3 g/day Level of evidence C NB! The presence of crescents significantly worsens the life and "renal" prognosis even with the timely start of induction therapy.
If active lupus nephritis is detected, in addition to the main therapy with GCs and cytostatics:	1. Plaquenil 200-400 mg/day - Reduced risk of exacerbations - Reduction of damage index and hypercoagulability; 2. Angiotensin receptor blockers (losartan 24-50 mg/day) - Reduce proteinuria by 30% - Reduce the risk of developing ESRD 3.Statins - Reduce LDL levels - Reduce the risk of developing cardiovascular complications
APS	Anticoagulants to prevent thrombosis (A) IN THE DEVELOPMENT OF CATASTROPHIC APS, high doses of HA, IVIG and plasmapheresis are additionally prescribed, which can reduce mortality (C) IF STANDARD THERAPY DOES NOT EFFECT, Rituximab or plasmapheresis can be used (C)

Drug treatment at the stage of emergency emergency care: provided by the relevant protocol for the provision of emergency emergency care.

Other types of treatment:

Other types of outpatient treatment: No.

Other types of treatment provided at the inpatient level: Plasmapheresis is designed to remove CEC from the blood, reduce serum levels of immunoglobulins G, inflammatory mediators , restoration of phagocytic activity.
Plasmapheresis sessions(PF) is advisable to be carried out as part of the so-called "synchronous therapy" - a combination of plasmapheresis sessions, pulse therapy with methylprednisolone and cyclophosphamide.
Indications for "synchronous therapy" are: SLE of high or crisis activity, accompanied by severe endogenous intoxication; highly active nephritis with renal insufficiency; severe damage to the central nervous system; lack of effect from combined pulse therapy with glucocorticoids and cyclophosphamide; the presence of APS resistant to standard therapy (LE - D).

Other types of treatment provided during the emergency phase: No.

Surgical intervention :

Surgical intervention provided on an outpatient basis: No.

Surgical intervention provided in a hospital:
Endoprosthesis replacement of joints - in case of severe damage to the musculoskeletal system (aseptic necrosis of the femoral head).

Treatment effectiveness indicators:
A patient is considered to have responded to therapy if the following changes are observed in the dynamics:
Decreased SELENA-SLEDAI Activity Index ≥ 4 points from baseline;
no new organ damage corresponding to BILAG class A, or the absence of two or more new signs of damage to an organ of BILAG class B compared to baseline;
no deterioration on the scale of the Global Assessment of the patient's condition by a doctor (an increase of no more than 0.3 points from the initial level is acceptable);

Drugs (active substances) used in the treatment

Azathioprine (Azathioprine)
Human albumin (Albumin human)
Atenolol (Atenolol)
Warfarin (Warfarin)
Heparin sodium (Heparin sodium)
Hydroxychloroquine (Hydroxychloroquine)
Dextran (Dextran)
Diclofenac (Diclofenac)
Ibuprofen (Ibuprofen)
Human normal immunoglobulin (Human normal immunoglobulin)
Captopril (Captopril)
Losartan (Losartan)
Methylprednisolone (Methylprednisolone)
Methotrexate (Methotrexate)
Mycophenolic acid (Mycophenolate mofetil) (Mycophenolic acid (Mycophenolate mofetil))
Naproxen (Naproxen)
Nimesulide (Nimesulide)
Nifedipine (Nifedipine)
Pentoxifylline (Pentoxifylline)
Plasma, fresh frozen
Rituximab (Rituximab)
Spironolactone (Spironolactone)
Fosinopril (Fosinopril)
Folic acid
Furosemide (Furosemide)
Cyclosporine (Cyclosporine)
Cyclophosphamide (Cyclophosphamide)
Enalapril (Enalapril)

Groups of drugs according to ATC used in the treatment

Hospitalization

Indications for hospitalization, indicating the type of hospitalization:
(planned, emergency) :

Indications for planned hospitalization in a round-the-clock hospital:
clarification of the diagnosis;
Monitoring the effectiveness of treatment and selection of immunosuppressive therapy;
Programmed planned pulse therapy to achieve remission induction;
· planned carrying out of genetically engineered biological therapy.

Indications for emergency hospitalization in a round-the-clock hospital:
Newly diagnosed SLE
SLE of any degree of activity;
secondary antiphospholipid syndrome;
Increased disease activity, complications of the disease and drug therapy;

Indications for treatment in a day hospital (PHC, day care beds in a round-the-clock hospital):
I and II degrees of SLE activity in chronic course;
planned continuation of subsequent infusions of GIBT.

Prevention

Preventive actions:
Primary prevention of SLEinvolves the identification and active monitoring of children at risk for this disease. Given the genetic predisposition, a group requiring special attention should include children whose close relatives suffer from SLE or other rheumatic diseases, including primary APS, as well as children with genetic defects in the complement system. These children, especially girls in puberty, should be recommended the same protective regimen as those with SLE: avoid excessive sun exposure, treatment with UVR and drugs that cause drug-induced lupus, etc. It is necessary to conduct periodic clinical and laboratory examinations for such children.
Secondary preventionis aimed at preventing relapses, progression of the disease and disability and includes a complex of therapeutic and recreational measures:
dispensary observation by a cardiorheumatologist;
regular clinical and laboratory-instrumental examination in order to identify the first signs of activation of the disease or complications of the treatment;
Carrying out anti-relapse therapy, including long-term and continuous use of corticosteroids in maintenance doses, if necessary, basic drugs and other drugs at recommended doses;
Compliance with the protective regimen: patients are advised to avoid insolation (do not sunbathe, stay outdoors for a long time), use sunscreens in the spring and summer, do not overcool or overheat, avoid physical and emotional stress; avoid exposure to UV radiation, contact with chemicals, food and household allergens; do not take any drugs without prescription, especially those that cause drug-induced lupus;
Establishment of an individual mode of study, taking into account the general condition (studying at home or at school, but with a reduction in the teaching load, if necessary, exemption from exams);
Sanitation of foci of chronic infection, taking into account the possible activation of tuberculosis infection, regular tuberculin tests;
withdrawal from vaccination and administration of sera (except for vital ones) in the active period of the disease; it is possible to vaccinate patients in the presence of indications only after they reach a state of remission, the question of the possibility of using live vaccines should be decided with great care.
Taking into account the clinical variant of the disease, paperwork on disability is carried out.

Further management:
All patients are subject to dispensary observation:
· timely recognize exacerbations of the disease and complications of drug therapy by monitoring the clinical and laboratory activity of SLE and preventing side effects of therapy through assessment.
Visiting a rheumatologist 2 times in 3 months (at least): every 3 months - UAC, OAM, BAC; annually: lipid profile study, densitometry, ophthalmological examination, determination of aPL titers (in the presence of secondary APS and pregnancy planning), radiography of the pelvic bones (detection of aseptic necrosis of the femoral head);
When prescribing high doses of corticosteroids and cytostatics in the induction phase, it is necessary to control the KLA, OAM, BAC 2 times a month (at least). When the effect is achieved and maintenance therapy is prescribed - 1 time in 2 months (at least). Upon reaching remission - 1 time per year;
The need for hospitalization of a patient with SLE is determined by a rheumatologist or emergency doctor; the duration and frequency of readmissions depends on the course, activity, and severity of SLE; repeated hospitalizations are justified in active LN; with multiple organ damage; in the presence of a large number of diagnostic criteria for SLE according to ACR; in case of failure of the aggressive therapy, when the control of the activity of the process is not achieved; with the development of complications associated with SLE and drug toxicity
· Evaluation of the SLE forecast.

Information

Sources and literature

Minutes of the meetings of the Expert Council of the RCHD MHSD RK, 2015
1. List of used literature: 1. WHO. International classification of diseases of the 10th revision. http://apps.who.int/classifications/icd10/browse/2015/en 2. Guidelines for pediatric rheumatology / ed. N.A. Geppe, N.S. Podchernyaeva, G.A. Lyskina M.: GEOTAR-Media, 2011 - pp. 333 - 393. 3. Rheumatology: national guidelines / ed. E.L. Nasonova, V.A. Nasonova. M.: GEOTAR-Media, 2010 - p. 478. 4. Podchernyaeva N. S. Systemic lupus erythematosus in children. Clinical guidelines. - M.: GEOTAR-Media, 2005.- 20 p. 5. Assessing remission in systemic lupus erythematosus. M. Mosca, S. Bombardieri. Clin Exp Rheumatol. 2006 Nov-Dec; 24(6 Suppl 43): S-99-104. 6. Podchernyaeva N. S. Systemic lupus erythematosus / Children's rheumatology: A guide for doctors / ed. A. A. Baranova, L. K. Bazhenova. - M.: Medicine, 2002.-S. 64-137. 7. EULAR recommendations for the management of systemic lupus erythematosus. Report of a Task Force of the EULAR Standing Committee for International Clinical Studies Including Therapeutics. G. Bertsias, J.P.A. Ioannidis, J. Boletis et. al. Annals of the Rheumatic Diseases, 2008; 67: 195-205 8. Hospitalization of individuals with systemic lupus erythematosus: characteristics and predictors of outcome. C J Edwards, T Y Lian, H Badsha, C L Teh, N Arden and H H Chng. Lupus 2003 12: 672 9. Abdellatif A. A., Waris S., Lakhani A. True vasculitis in lupus nephritis // Clin. Nephrol. - 2010; 74(2): 106-112. 10. Hiraki LT, Benseler SM, Tyrrell PN, Hebert D, Harvey E, Silverman ED. Clinical and laboratory characteristics and long-term outcome of pediatric systemic lupus erythematosus: a longitudinal study. J Pediatr, 2008;152:550-6. 11. Ho A, Barr SG, Magder LS, Petri M. A decrease in complement is associated with increased renal and hematologic activity in patients with systemic lupus erythematosus. Arthritis Rheum. 2001;44(10):2350–7. 12.E.A. Aseeva, S.K. Solovyov, E.L. Nasonov. Modern methods for assessing the activity of systemic lupus erythematosus. Scientific and practical rheumatology 2013; 51(2): 186–200. 13. Crow M. K. Developments in the clinical understanding of lupus // Arthritis Res. Ther. - 2009; 11(5): 245. 14. Ronald F van Vollenhoven. Et al. Treat-to-target in systemic lupus erythematosus: recommendations from an international task force. Ann Rheum Dis 2014; 00:1-10 doi: 10.1136/annrheumdis-2013-205139 15. Podchernyaeva N. S. Systemic lupus erythematosus In the book: Rational pharmacotherapy of childhood diseases: A guide for practicing physicians / ed. ed. A. A. Baranova, N. N. Volodina, G. A. Samsygina. - M.: Litterra, -2007, T. 1. - S. 878-902. 16. Systemic lupus erythematosus. In the book: Rational pharmacotherapy of rheumatic diseases / Guide for practitioners / ed. V. A. Nasonova and E. L. Nasonova. - M.: Literra, 2003. - 507 p. 17. Moore A., Deny S. Systematic review and meta-analysis of randomized trials and cohort studies of mycophenolate mofetil in lupus nephritis // Arthritis Res. and Ther. - 2006. - Vol. 8. - P. 182. 18. Ranchin B. Fargue S. New treatment strategies for proliferative lupus nephritis: keep children in mind: Rewiew // Lupus. - 2007. - Vol. 16. - P. 684-691. 19. Alekseeva E.I., Denisova R.V., Valieva S.I. RITUXIMAB IN PEDIATRIC RHEUMATOLOGY / Journal of Contemporary Pediatrics No. 3 / volume 9 / 2010 20. Grom AA. macrophage activation syndrome. In: Textbook of pediatric rheumatology. 6th ed. Cassidy JT, Petty RE, Laxer R, Lindsley C, editors. Philadelphia: Saunders, Elsevier; 2011:674–81. 21. Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: A potentially fatal complication of rheumatic disorders. Arch Dis LITERATURE 207 Continuing Postgraduate Education Program for Physicians Child. 2001;85(5):421–6. DOI 22. Dall "Era M., Wofsy D. Biologic therapy for systemic lupus erythematosus // Discov. Med. - 2010; 9 (44): 20-23. 23. Ranchin B. Fargue S. New treatment strategies for proliferative lupus nephritis: keep children in mind: Rewiew // Lupus. - 2007. - Vol. 16. - P. 684-691. 24. Treatment of the rheumatic diseases: Companion to Kelly's Textbook of Rheumatology / Eds. M.H. Weisman, M.E. Weinblatt, J.S. Louie. - 2nd ed. - W.B. Saunders Company, 2001. - 563 p. 25. European League Against Rheumatism recommendations for monitoring patients with systemic lupus erythematosus in clinical practice and in observational studies. M Mosca et al. Ann Rheum Dis. July 2010; 69(7): 1269–1274

Information

Developers:
1) Ishuova Pahitkanym Kabdukaevna - Doctor of Medical Sciences, Chief Researcher, Doctor of the Highest Category of the Department of Cardiorheumatology of the State Enterprise "Scientific Center of Pediatrics and Pediatric Surgery".
2) Maytbasova Raikhan Sadykpekovna - Doctor of Medical Sciences, Chief Researcher, Doctor of the Highest Category, Head of the Department of Cardiorheumatology of the State Enterprise "Scientific Center of Pediatrics and Pediatric Surgery".
3) Bugybai Aliya Aitbaevna. - Cardiorheumatologist, Department of Cardiorheumatology, State Enterprise "Scientific Center of Pediatrics and Pediatric Surgery".
4) Liya Ravilievna Litvinova - clinical pharmacologist of JSC "National Scientific Cardiac Surgery Center".

Conflict of interest: absent.

Reviewers:
1) Khabizhanov B.Kh. - Doctor of Medical Sciences, Professor of the Internship Department of the RSE on REM “Kazakh National Medical University named after S.D. Asfendiyarov".
2) Saatova G.M. - Doctor of Medical Sciences, Professor, Head of the Department of Rheumatology and Non-Rheumatic Heart Diseases of the "National Center for Maternal and Childhood Protection" of the Ministry of Health of the Kyrgyz Republic (Republic of Kyrgyzstan, Bishkek).

Conditions for revision of the protocol: revision of the protocol after 3 years and / or when new methods of diagnosis and / or treatment with a higher level of evidence appear.

Application

Monitoring activity SLE

According to the 2010 EULAR recommendations and the GCP rules, the following should be included in the standard examination of a patient with SLE in real clinical practice:
Assessment of disease activity using any validated SLE activity indices:
assessment of the degree of organ damage;
assessment of the patient's quality of life;
the presence of concomitant diseases;
drug toxicity.
Evaluation of SLE activity is of great importance for the choice of therapy. Monitoring of SLE activity at the present stage of development of rheumatology includes specially created tools - activity indices. All modern SLE activity indices, which are a combination of clinical and laboratory signs of lupus, were developed in order to standardize the assessment of disease activity, 5 SLE activity indices have been validated and are widely used in world medical treatment and scientific practice:
1. SLE Disease Activity Index (SLEDAI), (Bombardier et al. 1992)
2. Systemic Lupus Activity Measure (SLAM), (Liang et al. 1989)
3. European Consensus Lupus Activity Measurement (ECLAM), (Vitali et al. 1992)
4. Lupus Activity Index, (LAI) (Petri et al. 1992)
5. Classic British Isles Lupus Assessment Group Index (Classic BILAG) (Hay et al. 1993)

Systemic Lupus Erythematosus Disease activity score (SLEDAI) this index includes 24 parameters (16 clinical and 8 laboratory indicators of SLE). Each indicator was assigned scores from 1 to 8 for each of the SLE features included in the index. More severe manifestations of SLE, such as: nervous system damage, kidney damage, vasculitis, are scored higher than other signs. The total maximum possible score for the SLEDAI is 105 points. When assessing activity according to the SLEDAI index, it is necessary to note the signs of SLE that were present in the patient during the 10 days preceding the examination, regardless of their severity or improvement / worsening of the condition. A score > 20 is rare. An increase in SLEDAI > 8 indicates the presence of active disease. An increase in SLEDAI between two visits by >3 points is interpreted as a moderate exacerbation, by >12 points as a severe exacerbation of SLE. Currently, 3 modifications of the SLEDAI index are widely used: SLEDAI 2000 (SLEDAI 2K), SELENA-SLEDAI and Mex-SLEDAI. In clinical trials, the SELENA-SLEDAI index is more commonly used.
SELENA-SLEDAI, as well as SLEDAI 2K, takes into account persistent activity associated with the presence of rashes, mucosal ulcers and alopecia, and introduces the following changes: includes “dizziness” in “cranial nerve disorder”, makes changes to the sign “increase in proteinuria by 0 .5 g/day" for newly emerged, and allows to take into account only the presence of one of the signs of pleurisy or pericarditis, in contrast to the pre-existing need for a complex of symptoms. SELENA-SLEDAI, as well as SLEDAI 2K, takes into account persistent activity associated with the presence of rashes, mucosal ulcers and alopecia, and introduces the following changes: includes “dizziness” in “cranial nerve disorder”, makes changes to the sign “increase in proteinuria by 0 .5 g/day" for newly emerged, and allows to take into account only the presence of one of the signs of pleurisy or pericarditis, in contrast to the pre-existing need for a complex of symptoms.

Determination of SLE activity on a scaleSELENA- SLEDAI.
(Circle the score corresponding to the manifestation that occurred at the time of the examination or within 10 days preceding the examination).

score	Manifestation	Definition
8	epileptic seizure	Recent (last 10 days). Rule out metabolic, infectious, and drug causes
8	Psychosis	Violation of the ability to perform normal actions in a normal mode due to a pronounced change in the perception of reality, including including hallucinations, incoherence, a significant decrease in associative abilities, depletion of mental activity, pronounced illogical thinking; strange, disorganized, or catatonic behavior. Rule out similar conditions caused by uremia or drugs
8	Organic brain syndromes	Mental impairment with impairment of orientation, memory, or other intellectual abilities with acute onset and intermittent clinical manifestations, including blurred consciousness with reduced ability to concentrate and inability to pay attention to the environment, plus at least 2 of the following: impaired perception, incoherent speech, insomnia, or daytime drowsiness, decreased or increased psychomotor activity. Exclude metabolic, infectious and medicinal effects.
8	Visual disturbances	Changes in the eye or retina, including cell bodies, hemorrhage, serous exudate or hemorrhage in the choroid or optic neuritis, scleritis, episcleritis. Exclude cases of such changes with hypertension, infection and drug exposure.
8	Cranial Nerve Disorders	New onset sensory or motor neuropathy of the cranial nerves, including dizziness due to SLE.
8	Headache	Severe persistent headache (may be migraineous) not responding to narcotic analgesics
8	Violation of cerebral circulation	First appeared. Exclude that due to atherosclerosis or hypertension.
8	Vasculitis	Ulcers, gangrene, painful nodules on the fingers, periungual infarcts, and hemorrhages, or biopsy or angiogram evidence of vasculitis

4	Arthritis	More than 2 affected joints with signs of inflammation (tenderness, swelling, or effusion)
4	Myositis	Proximal muscle pain/weakness associated with elevated creatine phosphokinase/aldolase or EMG or biopsy evidence of myositis
4	Cylindruria	Granular or erythrocyte casts
4	Hematuria	>5 erythrocytes per field of view. Exclude urolithiasis, infectious and other causes
4	Proteinuria	Acute onset or recent appearance of protein in the urine in an amount > 0.5 grams per day
4	Piuria	>5 leukocytes per field of view. Rule out infectious causes
2	rashes	New or ongoing inflammatory skin rashes
2	Alopecia	New or ongoing increased focal or diffuse hair loss due to SLE activity
2	Mucosal ulcers	New or ongoing ulceration of the mucous membranes of the mouth and nose due to SLE activity
2	Pleurisy	Chest pain with pleural friction rub, or effusion, or pleural thickening due to SLE
2	Pericarditis	Pericardial pain with one of the following: pericardial friction rub, electrocardiographic confirmation of pericarditis
2	Low complement	Decrease in CH50, C3 or C4 below the limit of normal of the testing laboratory
2	Increasing the level of antibodies to DNA	>25% binding by the Farr method or above normal values of the testing laboratory
1	Fever	>38ºС. Rule out infectious causes
1	Thrombocytopenia	<100 000 клеток /мм 3
1	Leukopenia	<3000 клеток /мм 3 Исключить лекарственные причины
	Total score (the sum of the points of the marked manifestations)

SELENA Flare Index (SFI) The SELENA study defines the SELENA Flare Index (SFI) for the first time, with the help of which it becomes possible to differentiate the degree of SLE exacerbation into moderate and severe. SFI takes into account the dynamics of disease activity according to the SELENA SLEDAI scale, changes in the physician's global assessment of the patient's condition (physician's global-assessment visual-analogue scale, PGA), modification of treatment regimens, and a number of clinical parameters.
SELENA provides for the use of a general assessment of the patient's condition by a doctor, on a 100 mm visual analogue scale, but which is marked with gradations from 0 to 3 (where 0 means an inactive disease, and 3 means a disease with high activity). More recently, the term "SELENA SLEDAI Activity Score" includes the SELENA-SLEDAI Activity Score, Physician's VAS Overall Patient Assessment, and SFI Exacerbation Index.

systemicLupusErythematosusResponderIndex, SRIable to simultaneously detect improvements and deteriorations in the same and/or different organs and systems.

Index of response to SLE therapy,SRI
Your patient is considered a responder to therapy if the following principles are observed over time:

Index damageSLICC/ACR Damage Index
Establishes the presence of potentially irreversible lesions of various organs. The damage index includes a description of the state of 12 organ systems, the maximum score for individual organ systems is from 1 to 7 points, depending on the number of parameters evaluated. The total maximum possible score is 47 points. The scoring includes all types of damage since the onset of the disease (due directly to SLE or developed as a result of therapy), while taking into account only signs that persist for 6 months or more

SLE damage indexSLICC/ ACRdamage index.
(Patients must have had the following symptoms for at least 6 months.)

sign	Points
*Organ of vision (each eye) at clinical assessment*
Any cataract	1
Retinal changes or optic nerve atrophy	1
*Nervous system*
Cognitive impairment (memory decline, counting difficulties, poor concentration, difficulty speaking or writing, impaired performance) or major psychosis	1
Seizures requiring treatment for more than 6 months	1
Stroke ever (score 2 points if >1)	1 2
Cranial or peripheral neuropathy (excluding visual)	1
Transverse myelitis	1
*kidneys*
Glomerular filtration< 50 мл/мин	1
Proteinuria >3.5 g/24 hours	1
OR
End-stage kidney disease (regardless of dialysis or transplant)	3
*Lungs*
Pulmonary hypertension (right ventricular bulging or ringing II tone)	1
Pulmonary fibrosis (physical and radiological)	1
Shrunken lung (X-ray)	1
Pleural fibrosis (X-ray)	1
Lung infarction (X-ray)	1
*The cardiovascular system*
Angina pectoris or coronary artery bypass surgery	1
Ever myocardial infarction (score 2 if >1)	1 2
Cardiomyopathy (ventricular dysfunction)	1
Valvular disease (diastolic or systolic murmur >3/6)	1
Pericarditis within 6 months (or pericardectomy)	1
*peripheral vessels*
Intermittent claudication for 6 months	1
Slight loss of tissue ("pad" of the finger)	1
Ever significant tissue loss (loss of a finger or limb) (score 2 if > than at one site)	1 2
Venous thrombosis with edema, ulceration, or venous stasis	1
*Gastrointestinal tract*
Heart attack, bowel resection (below duodenum), spleen, liver, or gallbladder, ever for any reason (score 2 if more than one site)	1 2
mesenteric insufficiency	1
Chronic peritonitis	1
Strictures or surgery in the upper GI tract	1
*Musculoskeletal system*
Muscle atrophy or weakness	1
Deforming or erosive arthritis (including reducible deformities, excluding avascular necrosis)	1
Osteoporosis with fractures or vertebral collapse (excluding avascular necrosis)	1
Avascular necrosis (score 2 points if >1)	1 2
Osteomyelitis	1
*Leather*
Cicatricial chronic alopecia	1
Extensive scarring or panniculitis (other than scalp and fingertips)	1
Skin ulceration (excluding thrombosis) within 6 months	1
Damage to the reproductive system	1
Diabetes mellitus (regardless of treatment)	1
Malignancy (excluding dysplasia) (score 2 if more than one site)	1
Total score

Assessment of the quality of life (QOL).
The Short form Medical Outcomes Study (MOS SF-36) questionnaire is considered the standard for assessing QoL in patients with SLE. The Russian version of SF-36 was validated by the International Center for the Study of Quality of Life in St. Petersburg and Moscow. There is another, more specific questionnaire designed specifically to assess QoL in SLE patients. Lupus Quality of Life (LUPUSQOL). This is the only questionnaire translated into Russian by Corporate Translation Inc. according to all GCP rules.
Lupus-Qol is a questionnaire that includes 34 questions, united by 2-8 questions into separate scales. He evaluates: physical health (physical health); emotional health (emotional health); body image - body image (the patient's assessment of his body and its perception by others); pain (pain); planning (planning); fatigue (fatigue); intimate relationships (intimate relationships); burden to others (dependence on other people).

Lupus Quality of Life Questionnaire (LupusQoL) *Date VISIT Name Age years*
The questionnaire below is designed to measure how systemic lupus erythematosus (SLE) affects your life. Read each statement and mark the answer, most accurately reflects your well-being. Please try to answer all questions as truthfully as possible.
How often in the last 4 weeks
1. Due to lupus, I need help with heavy physical work, such as digging a vegetable garden, painting and/or redecorating, rearranging furniture	1 Constantly
	2 Almost always
	3 Quite often
	4 Occasionally
	5 Never
2. Due to lupus, I need help doing moderately heavy physical work, such as vacuuming, ironing, shopping, cleaning the bathroom	1 Constantly
	2 Almost always
	3 Quite often
	4 Occasionally
	5 Never
3. Due to lupus, I need help with light physical work such as cooking/cooking, opening jars, dusting, combing my hair, or doing personal hygiene.	1 Constantly
	2 Almost always
	3 Quite often
	4 Occasionally
	5 Never
4. Due to lupus, I am unable to perform daily activities, such as work, childcare, household chores, as well as I would like to.	1 Constantly
	2 Almost always
	3 Quite often
	4 Occasionally
	5 Never
5. I have difficulty climbing stairs due to lupus.	1 Constantly
	2 Almost always
	3 Quite often
	4 Occasionally
	5 Never
6. Due to lupus, I lost some of my independence and depended on other people	1 Constantly
	2 Almost always
	3 Quite often
	4 Occasionally
	5 Never
7. Lupus causes me to do everything slower.	1 Constantly
	2 Almost always
	3 Quite often
	4 Occasionally
	5 Never
8. I have a sleep disorder due to lupus.	1 Constantly
	2 Almost always
	3 Quite often
	4 Occasionally
	5 Never
9. Because of the pain caused by lupus, I can't do my job the way I would like to. The choice of drugs and their dosage should be discussed with a specialist. Only a doctor can prescribe the right medicine and its dosage, taking into account the disease and the condition of the patient's body. The MedElement website is an information and reference resource only. The information posted on this site should not be used to arbitrarily change the doctor's prescriptions. The editors of MedElement are not responsible for any damage to health or material damage resulting from the use of this site.

Systemic lupus erythematosus in children. Lupus erythematosus in children is a complex autoimmune disease.

Causes

Forms

Acute and subacute

Chronic

Symptoms

Diagnostics

Treatment

Complications

Prevention

Symptoms

Diagnostics

Treatment

Forms

Acute and subacute

Chronic

Symptoms

Diagnostics

Treatment

Complications

Prevention

Causes

Types of pathology

Characteristic signs and symptoms

Forms of lupus erythematosus in children

Diagnostics

Methods and general rules of treatment

Symptoms

Diagnostics

Treatment

The cause and mechanisms of the development of the disease

Symptoms of the disease

Damage to the musculoskeletal system

Mucosal and skin lesions

Respiratory damage

Damage to the cardiovascular system

Kidney damage

Damage to the central nervous system

Digestive tract injury

Diagnosis of SLE

Treatment of systemic lupus

What are the complications and prognosis for life with systemic lupus erythematosus?

Systemic lupus erythematosus and other systemic connective tissue diseases, how to differentiate?

Systemic lupus erythematosus in children, what are the features of symptoms and treatment?

Systemic lupus erythematosus and pregnancy, what are the risks and features of treatment?

Is lupus erythematosus contagious?

Lupus erythematosus, what diet is recommended and are there any methods of treatment with folk remedies?

Photo, what do the symptoms of lupus erythematosus look like?

General picture of the disease

Causes of Lupus

The first signs of lupus erythematosus in children

Medical diagnosis of lupus erythematosus

Symptoms of lupus erythematosus and forms of this disease in children

Acute course

Subacute course

chronic course

Which doctor should I contact and what treatment can he offer?

What to remember after reading the article

American scientists stepped forward in the treatment of lupus

general information

Short description

Classification

Clinical picture

Symptoms, course

Diagnostics

Laboratory diagnostics

Differential Diagnosis

Get treatment in Korea, Israel, Germany, USA

Treatment

Drugs (active substances) used in the treatment

Groups of drugs according to ATC used in the treatment

Hospitalization

Prevention

Information

Sources and literature

Information