Treatment of systemic lupus erythematosus. Systemic lupus erythematosus and other systemic connective tissue diseases, how to differentiate

Used to treat SLE:

2.Methods of intensive care,

3. Additional methods of pathogenetic therapy,

4. Auxiliary means.

For most patients with SLE modern therapy allows you to reduce the general signs of systemic inflammation, suppress most symptoms and syndromes, restore function individual bodies and systems.

The goal of treatment should be to achieve induced remission, which implies the absence of any clinical manifestations of SLE (in this case, there may be signs that have arisen due to lesions of one or another organ or system during previous exacerbations), the absence of cytopenic syndrome, and immunological examination of antinuclear antibody titers is minimal or not defined.

A. Evaluation of SLE activity:

1. Assessment of the overall activity of the disease: scales SLAM, SLEDAL (score score according to the severity of clinical manifestations and laboratory data)

2. The activity of nephritis is assessed taking into account: the level of daily proteinuria, urinary sediment, glomerular filtration.

B. Assessment of damage to internal organs and systems:

1.SLICC/ACR Damage Index (scoring the severity of signs of the disease)

2. Kidney damage: progression of nephritis to the stage of chronic renal failure requiring dialysis; doubling of serum creatinine, index of chronicity in histological examination of kidney tissue.

B. Evaluation of adverse drug reactions.

The main drugs for the treatment of SLE

Glucocorticosteroids for oral and intravenous administration

Immunosuppressants

Aminoquinoline derivatives

Glucocorticocteroids absolutely indicated for:

High inflammatory activity

Damage to internal organs, primarily nephritis,

CNS damage

hematological disorders.

The most commonly used drugs in rheumatological practice are: prednisolone, methylprednisolone, dexamethasone, polkortolone.

The appointment of an adequate dose of GC allows you to suppress most of the symptoms of the disease after 2-3 weeks from the start of therapy. Moreover, the initial dose of HA should be at least 40-60-80 mg of prednisolone per day - from 0.75 to 2 mg / (kg per day). The appointment of a lower dose of the drug, even with unexpressed manifestations of SLE, does not allow to achieve a positive result. With active lupus, higher doses of prednisolone (80-120 mg) can be used. The most effective is the use of GC inside, and the dose during the day is distributed as follows: in the first half of the day 2/3 of the selected dose, and in the evening 1/3. As soon as the regression of the main symptoms of the disease is observed, the dose of GCs begins to be reduced, but gradually, as a rule, canceled 1/2 tablet per week. Long-term administration of maintenance doses of GC (usually 5-15 mg of prednisolone per day) provides clinical and laboratory remission of the disease for many months and even years. However, in the treatment of mild forms of SLE, GCs may not be used.

Many side effects of GC are well known. Most often and earlier, complaints from the gastrointestinal tract may appear - pain in the upper abdomen due to the development of gastritis, duodenitis. GCs can cause ulceration of the mucous membrane of the stomach or duodenum. In this regard, patients are advised to take GC after meals and drink tablets with either milk or jelly. By following these simple rules, the risk of developing ulcers is significantly reduced. Ulcers in the gastrointestinal tract can become a source of internal bleeding. In such situations, liquid tarry stools are observed, which requires immediate medical attention.

Against the background of long-term use of GC, the development of Itsenko-Cushing's syndrome is possible, when there is a selective increased deposition of fat in the pelvic girdle and on the face in the cheek area, while the face becomes moon-shaped. Pink or cyanotic scars, the so-called stretch marks, appear on the lateral surfaces of the abdomen due to overstretching of the skin.

In elderly and middle-aged people, glucocorticoid intake can lead to the development of steroid diabetes. Depending on the level of hyperglycemia, it is recommended to follow a diet with a restriction of foods containing refined carbohydrates, or additional prescription of oral hypoglycemic drugs. As a rule, the transition to maintenance doses of GC contributes to the normalization of blood sugar.

It is known that glucocorticoids can cause osteoporosis when bone tissue is thinned and the risk of fractures increases. This complication can be avoided if, in order to prevent osteopenia, along with the appointment of GC, you start taking combined preparations of calcium and vitamin D.

Aminoquinoline drugs

Hydroxychloroquine (plaquenil) is the drug of choice in the treatment of SLE that occurs without damage to vital organs. An initial dose of 400 mg per day is good for reversing symptoms such as fatigue, arthralgias and skin lesions. At the same time, hydroxychloroquine helps to reduce the level of triglycerides and VLDL. Patients should be monitored quarterly using a slit lamp.

Cytotoxic immunosuppressants

In the absence of the expected effect from the use of GC against the background of the progression of the disease, with damage to vital organs (heart, kidneys, lungs, central nervous system), the question is raised about the appointment of second-line drugs in the treatment of SLE - cytostatic immunosuppressants. The most commonly used cytotoxic drugs are azathioprine or imuran, cyclophosphamide(1-2.5 mg per kg of body weight per day). Treatment with these drugs improves in more than half of SLE patients. Treatment with immunosuppressants gives better results when combined with GCs.

Discussions continue about the effectiveness of prednisolone alone, prednisolone in combination with cyclophosphamide or azathioprine in lupus nephritis. Currently, with III and IV morphological classes of lupus glomerulonephritis, it is considered appropriate to use methylprednisolone in combination with cyclophosphamide. Moreover, cyclophosphamide should be administered monthly in the form of pulse therapy at a dose of 1.0 g intravenously during the first 6 months. In the future, when remission is achieved, cyclophosphamide is administered less frequently (1 time in 2-3 months).

It should be remembered that in the treatment of cytostatics, various complications are possible - the development of leukopenia, agranulocytosis, anemia, thrombocytopenia. All these manifestations are associated with the toxic effect of this group of drugs on the hematopoietic organs. There is a need for careful hematological control - in particular, a blood test once every 3-4 weeks. The main way to stop these adverse reactions is to temporarily discontinue the drug or reduce the dose.

In recent years, a number of clinical studies have been conducted on the use of mycophenolate mofetil in the treatment of lupus nephritis. Also, the effectiveness of mycophenolate mofetil was demonstrated in a group of SLE patients with other extrarenal symptoms of the disease. The main effect of the drug is associated with blockade of the synthesis of guanosine, which leads to inhibition of the proliferation of T- and B-lymphocytes. The drug is prescribed 1000 mg 2 times a day. Unlike other cytostatic agents, mycophenolate mofetil is better tolerated.

In the absence of signs of kidney damage, it is possible to use methotrexate in small doses (7.5-15 mg per week). Methotrexate may also be used if antimalarial drugs do not respond.

Encouraging results in the treatment of SLE have been obtained with the use of a non-cytotoxic immunosuppressant - cyclosporine A, which is prescribed at a dose of 2.5-3 mg / (kg-day) orally for 6 months. However, its use may be limited in the development of arterial hypertension due to nephropathy.

Treatment of active forms of SLE

Treatment programs active forms SLE has its own characteristics due to the more aggressive course of the disease, which is accompanied by:

1) progressive course with the development of new symptoms and syndromes, despite the use of high doses of HA for 1-1.5 months; 2) lupus nephritis with the formation of nephrotic syndrome;

3) severe lesions of the central nervous system (acute psychosis, the appearance of focal symptoms, transverse myelitis, status epilepticus);

4) the development of life-threatening complications (exudative pericarditis, pneumonitis with increasing respiratory failure, recurrent thrombosis, etc.).

For getting therapeutic effect in the treatment of patients with SLE with high activity is used prednisolone at a dose of 2-3 mg / kg of body weight per day orally with its subsequent decrease.

The use of high doses methylprednisolone intravenously (1.0 g) for three to five consecutive days has become the standard treatment for patients with acute active lupus. When improvement is achieved after pulse therapy, repeated courses (once methylprednisolone intravenously up to 1 g) are possible every 3-4 weeks for 18 months. With the progression of nephritis or vasculitis, additional administration is required cyclophosphamide at a dose of 1000 mg intravenously on the first or last day of GC pulse therapy.

The infusion of drugs is carried out on a physiological solution slowly - for 30 minutes to an hour. Moreover, in some cases, such therapy can be carried out on an outpatient basis, subject to observation of the patient for 2-3 hours.

Some researchers have shown that intravenous use of smaller doses of methylprednisolone (500 mg) in some cases is not inferior in effectiveness to high doses. However, this provision does not apply to the treatment of lupus nephritis. The effectiveness of oral prednisolone in high doses is comparable to intravenous pulse therapy, but it is much cheaper and does not require hospitalization in some cases.

High doses of immunoglobulins.

Typically, intravenous administration of high doses of immunoglobulin is used for severe thrombocytopenia or immune neutropenia, as well as catastrophic antiphospholipid syndrome. The effectiveness of the use of immunoglobulins in the "non-hematological" manifestations of SLE remains doubtful.

Additional methods of pathogenetic therapy of SLE

Extracorporeal treatments for SLE.

In recent years, efferent methods of therapy have been widely used in the complex treatment of SLE: plasmapheresis, lymphocytopheresis, hemosorption, etc. Sorption and apheresis methods allow the removal of cell metabolism products, antibodies, and immune complexes from the body, which can deposit on the walls of blood vessels and cause inflammation. An important factor in extracorporeal methods of blood purification is an increase in the body's sensitivity to drugs and, first of all, HA. Plasmapheresis or plasma exchanges have proven themselves in the treatment of SLE that occurs with cryoglobulinemia, autoimmune thrombocytopenic purpura, DIC.

When planning individual therapy, one should take into account the nature of the course of the lupus process, the involvement of vital organs, the threat of complications, the degree of activity of the immunoinflammatory process. The doctor must remember that the treatment of SLE is not limited to the use of GCs and cytostatics. In table. groups indicated medicines, which can be used in the treatment of individual symptoms of the disease, as well as the feasibility of using physiotherapy and additional therapy for some manifestations of SLE.

Planning therapy for the main clinical manifestations of SLE

Manifestations of SLE	Analgesics	Steroids locally	Vascular drugs	Aminoquinoline agents	Glucocorticoids	Cytostatics	Physiotherapy	Complementary Therapy
Arthralgia
synovitis
tendonitis
Vasospasm
whining
Thrombosis
serosites
Pneumonitis

neuropathy
Cerebrovasculitis
convulsions

cytopenia
Dry syndrome
photosensitization

Symptomatic therapy

Non-steroidal anti-inflammatory drugs are a good adjunct to GC when moderate arthralgias occur, or the dose of the latter is being reduced.

Isolated seizures treated with anticonvulsants. Moreover, in some cases there is no need to increase the dose of GCS.

If chronic DIC is detected, as well as a tendency to recurrent thrombosis, long-term therapy with direct anticoagulants (heparin, fraxiparin) is indicated.

TO additional therapy include antidepressants, migraine medications, phenothiazines.

In the treatment of autoimmune thrombocytopenia, gamma globulin is used intravenously.

The efficacy of splenectomy for severe thrombocytopenia in SLE patients is debated.

Calcium channel blockers (nifedipine) are used in the treatment of Raynaud's syndrome.

With the development of severe tissue ischemia, vasodilators with antithrombotic potential (intravenous prostacyclin) are indicated.

Prevention

Preventive measures aimed at preventing exacerbations:

For patients with photosensitivity, direct contact should be avoided. sun rays;

Regular exercise can reduce muscle weakness;

Avoid smoking, alcohol abuse.

History of study systemic lupus erythematosus can be divided into three periods: classical, neoclassical and modern. The disease was first described in the 12th century by Rogerius, who was the first to use the term "lupus" to describe the classic red rash on the face. The next stage is directly related to the name of Kaposi, who in 1872 noted the presence systemic manifestations(that is, lesions of many organs). In 1948, lupus cells (LE-cells or LE-cells in Russian transcription) were discovered, the second half of the 20th and the beginning of the 21st century became a period of active study of the mechanisms of the development of the disease and tremendous progress in treatment.

It's interesting that lupus has a lot common symptoms with porphyrias - rare diseases associated with impaired pigment metabolism. It is believed that it was patients with porphyria who became the prototype for the appearance of stories about vampires and werewolves (they often have photophobia, red staining of teeth, overgrowth hair and other "terrible" symptoms). It is possible that lupus patients also contributed to the formation of such folklore.

The most famous patient systemic lupus erythematosus— Michael Jackson, who fell ill in 1984.

General information about systemic lupus erythematosus

Systemic lupus erythematosus (SLE or just lupus)- chronic autoimmune disease which can affect the skin, joints, kidneys, lungs, nervous system and/or other organs of the body.

The severity of symptoms can vary. Systemic lupus erythematosus women are more often affected. The disease usually proceeds in the form of alternating periods of deterioration and improvement, but improvement usually occurs only through proper treatment.

Special attention should be paid to special occasions lupus that is different from systemic lupus erythematosus:

Discoid lupus erythematosus (cutaneous lupus) — chronic illness skin, in which a rash forms, and subsequently - scars. Rashes can appear from several days to several years, relapses are possible. The development of systemic lupus erythematosus occurs only in a small number of cases.
Medicinal lupus symptoms are similar to systemic lupus erythematosus, but the kidneys and brain are rarely affected. The main difference lies in known reason diseases: it is associated with taking medications. Most often, these are Hydralazine, Isoniazid, Methyldopa, Minocycline and some others. When the drug is discontinued, the disease usually goes away on its own.
Neonatal lupus develops in a newborn if special antibodies from a sick mother enter his blood. It is most often manifested by a skin rash that resolves on its own in a period of up to 6 months. The main complication is cardiac arrhythmias. It should be remembered that neonatal lupus rarely develops. Most often when proper planning pregnancy in women with systemic lupus erythematosus, healthy children are born.

On our site you will also find information about such a disease as fibrous mastopathy.

Symptoms of systemic lupus erythematosus

Symptoms of systemic lupus erythematosus are very diverse. With this disease, lesions of almost all organs are possible, while the variants of the lesion are also different. Each individual patient may experience different combinations of symptoms. The severity of symptoms of systemic lupus erythematosus also fluctuates.

Increased body temperature, general feeling of malaise, weight loss.
Inflammation of the joints, manifested by pain in them, swelling and redness.
Butterfly shaped rash on cheeks and bridge of nose.
A rash on other parts of the body that appears or worsens after exposure to the sun.
Formation of ulcers in the mouth.
Hair loss.
Episodes of loss of consciousness, disorientation.
Shortness of breath, muscle pain, weakness, dry mouth and eyes, and many others.

Symptoms of systemic lupus erythematosus may increase, disappear for a while or remain at the same level, so it is important to suspect the possibility of this disease in advance and conduct a proper follow-up examination.

Causes of systemic lupus erythematosus

The cause of systemic lupus erythematosus is unknown. Genetic, immunological, hormonal and external factors take part in its development. Some patients trigger factor diseases become viral infections.
The basis of the disease is a violation of work immune system. As a result of a breakdown, the immune system begins to produce special proteins (antibodies) that attack normal tissues and organs. As a result, inflammation develops in them, leading to damage.
Risk factors for systemic lupus erythematosus

The risk of systemic lupus erythematosus is increased if relatives have autoimmune diseases. Women are more often ill, especially at the age of 20-40 years.

Prevention of systemic lupus erythematosus

There are no effective methods of prevention.

Diagnosis of systemic lupus erythematosus

For the diagnosis of Sjögren's syndrome, the correct questioning of the doctor, as well as blood tests, is of great importance.
There are no specific diagnostic tests.
In blood tests, the presence of inflammation is assessed, and special antibodies are also determined. Most characteristic changes: increase in ESR(erythrocyte sedimentation rate), C-reactive protein (CRP), antinuclear factor (ANF). Very often there is a decrease in the number of blood cells (erythrocytes and hemoglobin - anemia, leukocytes - leukopenia, platelets - thrombocytopenia).
It is mandatory to study the condition of the kidneys (urinalysis, determination of the level of serum creatinine), since kidney damage is serious threat health and life and necessitates active and aggressive therapy.
Often, a study is needed for specific antibodies that can increase with systemic lupus erythematosus - antibodies to DNA, anti-Ro (anti-ro) and anti-La (anti-la) antibodies, antibodies to the Sm antigen.
You may need a biopsy of the skin, kidneys, which allow you to clarify the nature of organ damage.
Depending on the damage to other organs and tissues, the most various analyzes and research

Treatment of systemic lupus erythematosus

Treatment for systemic lupus erythematosus can make you feel better and help prevent internal organ damage and, if affected, slow or stop its progression.
Importance belongs to the protection from direct sunlight. It is necessary to use sunscreen with a high degree of protection, try not to be under the sun long time.
IN active phase disease physical activity should be avoided, during remission the level physical activity may be normal.
It is necessary to avoid infections, vaccinations, taking various biological active additives because they can exacerbate the disease.
The question of the appointment of glucocorticoid drugs and cytostatics is decided by the doctor. In most cases, they well remove the symptoms of the disease and prevent the development of severe consequences. However, the side effects of glucocorticoids are very serious, the question of their appointment, dose and duration of therapy is decided by the doctor individually. Immunosuppressive drugs (from hydroxychloroquine to cyclophosphamide) are also commonly used to reduce the dose or eliminate glucocorticoids in the future. The choice of specific drugs and treatment regimens is very individual.

Systemic lupus erythematosus, otherwise known as Limban-Sachs disease, is an autoimmune connective tissue disorder that primarily affects young women and children. The development of the disease is based on a malfunction of T-lymphocytes, important cells of the immune system. Up to 90% of all patients are women under the age of thirty. Systemic lupus erythematosus in children is most often diagnosed in adolescence(the peak of this disease in children occurs at 11-14 years old), is less often found in children of the first years of life. The causes of the disease are measles and parainfluenza viruses. There is also a hereditary factor in lupus. Excessive sun exposure and the use of certain vaccines in a child contribute to the aggravation of the disease. In women, the risk of developing lupus increases after childbirth and abortion, due to malfunctions in the production of the hormones estrogen and prolactin. In children, the risk of morbidity increases during periods of active physical growth.

The course of the disease systemic lupus erythematosus and its clinical manifestations

Systemic lupus erythematosus initially has vague symptoms: fever, headache, muscle cramps, nervousness, bad dream sometimes diarrhea. Then there are specific characteristic skin and joint manifestations:

a classic sign - lupus "butterfly" - redness of the skin and a rash on the bridge of the nose and cheekbones, a little less often - on the earlobes, neck and scalp, even less often - on the body;
hemorrhagic rash on the palms and fingertips, caused by bursting of the smallest vessels;
small painful ulcers in the throat, nose, lips;
brittle nails and dry hair, tufted hair loss;
aching pains in the knees, hands, coccyx and sacrum;
the connective articular tissue is destroyed and polyarthritis inflammations appear in the joints.

The skin symptoms of lupus are markedly aggravated by low temperatures(in winter) or vice versa, with intense sunburn, as well as with psycho-emotional shocks.

The disease is steadily progressive in nature, so over time, its effects spread to the entire body as a whole. The systemic disease lupus erythematosus affects whole line organs and systems of the patient:

joints (lupus arthritis of the joints of the hands and ankles);
cardiovascular system (pericarditis and endocardium, damage to the heart valves, high probability of atherosclerosis);
gastrointestinal tract (hemorrhages in intestinal walls, dyspeptic disorders):
kidneys (lupus nephritis, blood and high content squirrel);
nervous system (half of patients suffer from depression, headaches, sleep problems).

The acute form of the disease, systemic lupus erythematosus, begins rapidly with a rise in temperature and the appearance of a "lupus" butterfly on the face. Within one and a half to two months, a full picture damage to internal organs. The prognosis is unfavorable.

The subacute form of the disease, systemic lupus, does not have a sudden onset, it develops gradually and, as a rule, the first complaints of the patient are joint pains, and then only skin rashes. On average, systemic lupus erythematosus syndrome is fully formed in 1.5-2 years and continues to progress at a rapid pace. The insufficiency of the functions of any organs or the secondary infection that has joined, bedsores and trophic ulcers can lead to death.

The chronic course of systemic lupus erythematosus is manifested by one or two symptoms during the first years. Exacerbations are rare, vital important organs practically unaffected.

Diagnostics

When systemic lupus erythematosus is diagnosed, the diagnosis is quite simple. The diagnosis is made on the basis obvious symptoms(minimum 4 characteristic feature) And laboratory research. The main analysis for lupus is a test for "lupus cells" - an excess of specific LE cells is found in the blood, directly indicating the presence of the disease. In addition, the study of skin cells is carried out.

If there is a suspicion of damage to the internal organs, an x-ray of diseased joints, ultrasound of the heart and abdominal cavity, ECG of the heart, determine respiratory function lungs. Diagnosis of systemic lupus erythematosus should be timely, as this is directly related to treatment, which must be started immediately.

Treatment of systemic lupus erythematosus

The treatment of the disease is effective in early stages at obviously severe symptoms. At each exacerbation, the patient is placed in stationary conditions. Systemic lupus erythematosus involves treatment with drugs of various directions, depending on the predominance of certain clinical signs.

If joint symptoms predominate, then therapy is started with salicylates (Aspirin and Analgin) and non-steroidal anti-inflammatory drugs (Ibuprofen, Indomethacin), which reduce inflammation and relieve joint pain. If the patient has predominantly skin symptoms, then prescribe drugs of the chonoline series (Chloroquine, Rezokhin, Delagil). Both therapies are long-term, lasting at least six months. More long reception Such drugs give many side effects, the manifestations of which are nausea, vomiting, loss of appetite, loss of vision. However, these side effects are short-lived and disappear after stopping the medication.

Mandatory in systemic lupus erythematosus is glucocorticoid therapy. Its use is most justified when the pathology spreads to the heart, kidneys and nervous system. Prednisolone remains the drug of choice. If the patient's body shows resistance to Prednisolone, then it is replaced with Dexamethasone. Long term treatment glucocorticoids leads to the development of hypertension, weakening muscle tone. Glucocorticoid therapy begins with shock maximum doses, and when the patient feels better, the dose is gradually reduced. A complication of such therapy can be gastrointestinal disorders, as well as the occurrence of peptic ulcers of the stomach due to hyperacidity gastric juice. Therefore, with systemic lupus, a strict diet is prescribed with the exception of sauces, spices, marinades and other irritating dishes.

To improve the condition of the connective tissue, patients are shown potassium preparations, B vitamins and their combinations in combination with vitamin A and C. Prevention of osteoporosis is carried out with calcium preparations together with vitamin D. If there are foci of chronic infection, then antibiotics are indicated. The immunosuppressive effect is achieved through the use of systemic cytostatics (Cyclophosphamide, Methotrexate, Cyclosporine). They are prescribed for deep lesions of the kidneys or nervous system.

To relieve itching in systemic lupus erythematosus is used hormonal ointments(Betamethasone, Celestoderm). In some cases, the affected skin areas are cut off with hormonal preparations.

Skin manifestations, redness and itching can be relieved by some folk ways treatment. For example, rubbing the skin homemade ointment prepared as follows: 200 ml of olive oil, 1 tablespoon of violet herb and 1 tablespoon of string are mixed and kept in a water bath.

Other effective tool for removal skin inflammation with lupus, a decoction of licorice root is used, since this plant contains hormone-like substances in its composition.

Forecast and prevention of lupus

Systemic lupus erythematosus has a different prognosis depending on the severity of the destruction of internal organs, the duration of remissions and the timeliness of the treatment started. Therefore, timely diagnosis of the disease is very important. Drug therapy well relieves all symptoms systemic lupus and with the right treatment regimen, if it is possible to achieve persistent remissions, the patient is predicted another 8-12 years of life. However severe forms systemic lupus erythematosus are unfavorable and the patient may die within the first three years after diagnosis. lethal outcome contribute to damage to the nervous system, kidneys, brain (meningitis), as well as the onset pulmonary bleeding. Concomitant pathologies in the liver (fibrous lesions) and vasculitis of the coronary arteries lead to disability in lupus.

There is no direct prevention of the disease. Doctors advise less exposure to direct sunlight, avoid skin contact with chemicals to minimize skin injury. If close relatives, especially in the female line, have lupus, then it is recommended to strictly monitor the child for the slightest skin rashes and in case of their appearance immediately contact for qualified help. In patients with systemic lupus erythematosus, prevention during periods of remission is especially important, aimed at lengthening their period.

Rebrov A.P.
MD Prof., Head of the Department of Hospital Therapy, Faculty of Medicine, Saratov State Medical University (SSMU).

Systemic lupus erythematosus - a disease that develops on the basis of a genetically determined imperfection of immunoregulatory processes, leading to the formation of immune complex inflammation, which results in damage to many organs and systems.

The frequency of SLE is 4-250 cases per 100,000 population per year. In the US, the annual incidence of SLE is 50-70 new cases per 1 million population.

More than 70 percent fall ill at the age of 14-40 years, the peak of incidence falls on 14-25 years. The ratio of women and men is from 8:1 to 10:1, among children - 3:1.

Etiology

The starting role of RNA-containing and slow viruses (retroviruses):
1. formation of antibodies to DNA and RNA-containing viruses,
2. the presence of paramyxovirus cytoplasmic inclusions,
3. the presence of tubuloreticular structures in the epithelium and within lymphocytes,
4. inclusions of C-oncornovirus type in kidney and skin biopsy.

Matter:
1. genetic factors (HLA-A1, B8, DR2, DR3),
2. endocrine factors (effect of estrogens),
3. environmental factors (ultraviolet radiation, exposure to bacterial and viral infection, pharmaceuticals).

Systemic lupus erythematosus is an immune complex disease characterized by uncontrolled production of antibodies that form immune complexes that cause various signs illness.

CECs are deposited in the subendothelial layer of the basement membrane of vessels in many organs.

The place of fixation of deposits (skin, kidneys, choroid plexus, serous membranes) is determined by such antigen or antibody parameters as size, charge, molecular configuration, class of immunoglobulins, etc.

Clinical picture
Skin lesions - very diverse, in 20-25% skin syndrome- the initial sign of illness, in 60-70% - appears on different stages diseases.

There are 28 options skin changes in SLE from erythematous patches to severe bullous eruptions.

Damage to the joints and periarticular tissues - arthralgia in 100% of patients, tendinitis, tendovaginitis, aseptic necrosis bones - in 25% of patients.

Myalgia - in 35 - 45% of patients.

Lung damage:
1. in 50-80% - dry and effusion pleurisy,
2. vasculitis,
3. pneumonitis.

Damage to the heart and blood vessels
1. pericarditis - often dry, effusion,
2. myocarditis,
3. endocarditis - more often mitral, also aortic, tricuspid valve,
4. arteries of medium and small caliber,
5. aorta and its branches,
6. thrombosis of the main vessels of the extremities,
7. thrombophlebitis

Damage to the gastrointestinal tract and liver - in 50% of cases:

Damage to the esophagus - 10-15%, ischemia of the wall of the stomach and intestines, hepatomegaly - 25-50%.

Kidney damage

Lupus nephritis: active forms
1. rapidly progressive,
2. nephritis with nephrotic syndrome,
3. nephritis with severe nephritic syndrome

Nephritis with minimal urinary syndrome

Damage to the nervous system
1. vasculopathy - 65%,
2. thrombosis and true vasculitis - 15%,
3. heart attacks and hemorrhages,
4. antibody and immunocomplex lesion

Clinical manifestations:
1. headache,
2. mental disorders
3. damage to the cranial and peripheral nerves,
4. seizures,
5. visual disturbances,
6. transient disorders of cerebral circulation.

Laboratory research
1. LE cells - 50-80%,
2. antinuclear antibodies,
3. antibodies to double-stranded DNA - 50%,
4. antibodies to single-stranded DNA - 60-70%.
Anemia normocytic and normochromic, leukopenia, lymphocytopenia, thrombocytopenia.

SLE classification
Course variant: acute, subacute, chronic,

Degree of activity: I - minimal, II - moderate, III - high

Criteria for diagnosing systemic lupus erythematosus (American Association of Rheumatologists, 1982.).

Erythema on the cheeks, over the zygomatic prominences, Discoid lesions of lupus, Photosensitivity, Ulcers in the mouth or nose, Non-erosive arthritis, Pleurisy or pericarditis, Persistent proteinuria more than 0.5 g per day or changes in urinary sediment, Convulsions and psychosis, Hemolytic anemia or leukopenia or thrombocytopenia, Presence of LE cells or anti-DNA or SM antibodies or false positive Wasserman reaction, Presence of ANF.

The presence of 4 signs makes the diagnosis reliable.

To establish the diagnosis of SLE, young age, female sex, persistent fever, significant and fast loss body weight, increased hair loss.

Treatment of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune disease whose pathogenesis is based on immunoregulatory defects leading to uncontrolled hyperproduction of autoantibodies to components of one's own tissues and the development of chronic inflammation affecting many organs and systems.

Used to treat SLE:
1. The main methods of pathogenetic therapy,
2. Methods of intensive care,
3. Additional methods of pathogenetic therapy,
4. Auxiliaries

Absolute indications for treatment with glucocorticosteroids in SLE:
1. High inflammatory activity,
2. Damage to internal organs, primarily nephritis,
3. Damage to the central nervous system,
4. Hematological disorders.

A suppressive dose of prednisolone 1-1.5 mg/kg per day, on average about 60 mg/day, for 4-8 weeks with a gradual decrease to a maintenance dose of 5-10 mg/day, which is taken for a long time, often for life, moving from doses of prednisolone 60 mg / day to a dose of 35-40 mg / day takes 3 months, and to a dose of 15-20 mg / day - 6 months.

The main drugs for the treatment of SLE

Glucocorticosteroids for oral administration . The most commonly used are prednisolone, methylprednisolone (metipred, medrol), rarely used or as an alternative - triamcinolone.

Glucocorticosteroids for intravenous administration (pulse therapy). The most commonly used are methylprednisolone (metipred, solumedrol, urbazone).

Immunosuppressants. The most commonly used are cyclophosphamide (cyclophosphamide), azathioprine (imuran). Used rarely or as an alternative - chlorambucil (chlorbutine), methotrexate, cyclosporine A (sandimmune).

Aminoquinoline derivatives . The most commonly used is hydroxychloroquine (plaquenil). They are rarely used or as an alternative - chloroquine (delagil).

Schemes for the use of essential drugs for the treatment of SLE

Prednisone inside.
1. suppressive therapy - 1-1.5 mg / kg / day (average 50-60 mg / day) 4-8 weeks,
2. maintenance therapy - 5-10 mg / day (10-15 years, often for life).

Methylprednisolone intravenously.
1. suppressive therapy - 500-1000 mg in accordance with the intensive care regimen,
2. maintenance therapy - 500-1000 mg once a month (up to 24 months).

Cyclophosphamide intravenously.
1. suppressive therapy - 500 mg once a week for 4 weeks or 1000 mg 1-2 times in combination therapy or 200 mg every other day 10 times (up to a total dose of 2000 mg per month).
2. maintenance therapy - 1000 mg once a month for 6 months, then 200 mg once a week with an increase in the interval between injections (up to 5 years).

Azathioprine
1. suppressive therapy - 100-150 mg / day,
2. maintenance therapy - 50-100 mg / day (up to 5 years).

Hydroxychloroquine.
1. suppressive therapy - 600 mg / day,
2. maintenance therapy - 200-400 mg / day (long-term, often for life).

Intensive Care Systemic Lupus Erythematosus

The main indications for the use of pulse therapy:

Active lupus nephritis (especially with nephrotic syndrome, hypertension, rapid rise creatinine level), Acute severe CNS injury (meningoencephalitis,is, transverse myelitis), Hematological crisis, deep thrombocytopenia, Ulcerative necrotic cutaneous vasculitis, Pulmonary vasculitis, high activity disease resistant to therapy.

The main method of intensive therapy for systemic lupus erythematosus - pulse therapy - is performed with methylprednisolone at a dose of 500-1000 mg / day intravenously.

Doses of less than 1000 mg of methylprednisolone per day are used at an increased risk of side effects - in elderly patients, in the presence of high arterial hypertension, severe heart failure, etc.

Less often, dexamethasone is used at an average dose of 100-150 mg per day according to various schemes.

It is advisable to use the following schemes:

Monthly administration of 1000 mg of methylprednisolone for 1 year, Combined (with the addition of 1000 mg of cyclophosphamide) pulse therapy, both three-day and program during the year.

The most common methods of intensive care:

Classic pulse therapy of 1000 mg methylprednisolone per day intravenously for 3 consecutive days (3000 mg per course), Intravenous administration of reduced doses of methylprednisolone (250-500 mg/day) until a total dose of about 3000 mg per course is reached, Monthly intravenous 1000 mg methylprednisolone for 6-12 months, Combination pulse therapy IV 1000 mg methylprednisolone on 3 consecutive days + 1000 mg cyclophosphamide on the 1st or 2nd day (methylprednisolone and cyclophosphamide are administered sequentially), Monthly IV introduction of 1000 mg of methylprednisolone + 1000 cyclophosphamide for 12 months, Monthly IV administration of 1000 mg of cyclophosphamide for 12 months.

It is not recommended to reduce the dose of oral prednisolone immediately after pulse therapy with glucocorticosteroids (a temporary withdrawal syndrome is possible).

Additional methods of pathogenetic therapy of SLE

Plasmapheresis is the method of choice for acute conditions and extremely high disease activity, resistance to therapy.

Plasmapheresis is carried out in a course of 3-6 procedures every other day or 2 times a week, as well as programmatically - 1 time per month monthly for a year or more, and in order to avoid the rebound syndrome, it is always combined with subsequent intravenous administration of glucocorticoids and cyclophosphamide.

Synchronous intensive therapy: plasmapheresis in a course (3-6 procedures) followed by combined pulse therapy with glucocorticoids and cyclophosphamide.

Immediately after the first plasmapheresis procedure, a sequential injection of 1000 mg of methylprednisolone and 1000 mg of cyclophosphamide is performed, after repeated sessions of plasmapheresis during the course of treatment, only methylprednisolone is administered intravenously at a dose of 500-1000 mg.

Synchronous intensive care may also be given monthly for 12 months or more.

Intravenous administration of immunoglobulin (sandoglobulin, normal human immunoglobulin): Blockade of FC receptors and FC-dependent synthesis of autoantibodies, Anti-idiotypic activity, Modulation of T-lymphocyte activity and cytokine synthesis, Changes in the structure and solubility of circulating immune complexes.

The use of intravenous immunoglobulins is the method of choice for severe persistent thrombocytopenia, with resistance to lupus nephritis therapy. It is recommended to administer the drug at a dose of 400-500 mg / kg per day for 3-5 consecutive days. then once a month for 6-12 months.

Cyclosporin A - the mechanism of action in SLE is associated with inhibition of the synthesis of interferon-alpha and is able to suppress the expression of the CD40 ligand on the membrane of T-lymphocytes.

In SLE, low doses of cyclosporine A are used (less than 5 mg / kg / day, more often 2-2.5 mg / kg / day). Efficacy is shown in lupusnephritis (pronounced antiproteinuric effect), thrombocytopenia. anemia and leukopenia, skin manifestations of SLE, polyserositis refractory to therapy and arthritis. During therapy with cyclosporine A, the level of anticardiolipin and antiplatelet antibodies decreases.

Cyclosporin A - alternative drug second row with intolerance and ineffectiveness of glucocorticosteroids and cytostatics. Cyclosporine A can be prescribed during pregnancy.

Myofetil Mycofelate(CellCept) is a selective immunosuppressant. The active compound, mycophenolic acid, is a non-competitive inhibitor of an enzyme that limits the rate of synthesis of guazine nucleatides and exhibits cytostatic rather than cytotoxic activity.

A more pronounced antiproliferative effect on T- and B-lymphocytes, has an antiproliferative effect on mesangial cells of the kidneys, and inhibits the formation of antibodies.

In SLE patients with kidney damage - an alternative to azathioprine and cyclophosphamide with better tolerance.

"Biological agents" - anti-idiotypic monoclonal antibodies, intravenous immunoglobulin, monoclonal antibodies to IL-10.

Autologous stem cell transplant.

systemic lupus erythematosus- a chronic polysyndromic disease of the connective tissue and blood vessels, which develops due to a genetically determined imperfection of immunoregulatory processes.

Etiology. The importance of a viral infection against the background of genetically determined immunity disorders is assumed.

Pathogenesis: the formation of circulating autoantibodies, of which antinuclear antibodies have the most important diagnostic and pathogenetic significance; the formation of circulating immune complexes, which, being deposited on the basal membranes of various organs, cause their damage and inflammation. Such is the pathogenesis of nephritis, dermatitis, vasculitis, etc. This hyperreactivity humoral immunity associated with disorders of cellular immunoregulation. Recently, importance has been attached to hyperestrogenemia, accompanied by a decrease in the clearance of circulating immune complexes, etc. A family genetic predisposition has been proven. Mostly young women and teenage girls are ill. Provocative factors are: insolation, pregnancy, abortion, childbirth, the onset of menstrual function, infections (especially in adolescents), drug or post-vaccination reactions.

Symptoms, course. The disease begins gradually with recurrent polyarthritis, asthenia. Less often there is an acute onset (high fever, dermatitis, acute polyarthritis). In the future, there is a recurrent course and characteristic polysyndromicity.

Polyarthritis, polyarthralgia- the most common and early symptom diseases. Mainly affected small joints hands, wrist, ankle, rarely knee joints. A non-erosive type of polyarthritis is characteristic, even in the presence of deformity of the interphalangeal joints, which develops in 10-15% of patients with a chronic course. Erythematous rashes on the skin of the face in the form of a "butterfly", in the upper half chest in the form of a "neckline", on the limbs - also frequent sign systemic lupus erythematosus. Polyserositis is considered a component of the diagnostic triad along with dermatitis and polyarthritis. It is observed in almost all patients in the form of bilateral pleurisy and (or) pericarditis, less often perihepatitis and (or) perisplenitis.

Defeat is characteristic of cardio-vascular system. Usually pericarditis develops, to which myocarditis joins. Relatively common observed warty endocarditis Libman-Sachs with damage to the mitral, aortic and tricuspid valves. Signs of vascular damage are included in the picture of damage to individual organs. Nevertheless, it should be noted the possibility of developing Raynaud's syndrome (long before the typical picture of the disease), the defeat of both small and large vessels with corresponding clinical symptoms.

Lung lesions may be associated with the underlying disease in the form of lupus pneumonitis, characterized by cough, shortness of breath, unvoiced moist rales in the lower parts of the lungs. X-ray examination in such patients reveals an increase and deformation of the pulmonary pattern in the basal sections of the lungs; at times, focal-like tones can be detected. Since pneumonitis usually develops against the background of current polyserositis, the described radiological symptoms are supplemented by a high standing of the diaphragm with signs of pleurodiaphragmatic and pleuropericardial adhesions and discoid atelectasis (linear shadows parallel to the diaphragm).

In the study of the gastrointestinal tract aphthous stomatitis, dyspeptic syndrome and anorexia are noted. painful abdominal syndrome it can be associated both with the involvement of the peritoneum in the pathological process, and with vasculitis proper - mesenteric, splenic, etc. Segmental ileitis develops less frequently. The defeat of the reticuloendothelial system is expressed in an increase in all groups lymph nodes- a very frequent and early sign of the systemic nature of the disease, as well as an increase in the liver and spleen. Actually lupus hepatitis develops extremely seldom. However, liver enlargement may be due to heart failure with pancarditis or severe effusion pericarditis, as well as the development of fatty liver.

Lupus diffuse glomerulonephritis(lupus nephritis) develops in half of the patients, usually during the period of generalization of the process. Meet various options kidney damage- urinary syndrome, nephritic and nephrotic. For the recognition of lupus nephritis, intravital puncture biopsy with immunomorphological and electron microscopic examination of the kidney biopsy is of great importance. The development of renal pathology in patients with recurrent articular syndrome, fever and elevated ESR requires the exclusion of the lupus genesis of nephritis. It should be remembered that almost every fifth patient with nephrotic syndrome has systemic lupus erythematosus.

Defeat of the neuropsychic sphere occurs in many patients in all phases of the disease. At the onset of the disease - asthenovegetative syndrome, subsequently develop signs of damage to all parts of the central and peripheral nervous system in the form of encephalitis, myelitis, polyneuritis. Damage to the nervous system in the form of meningoenis characteristic. Epileptiform seizures are less common. Hallucinations (auditory or visual), delusional states, etc. are possible.

Laboratory data have diagnostic value: definition a large number LE cells, high titers of antibodies to DNA, especially to native DNA, to deoxyribonucleoprotein, Sm antigen.

In an acute course, lupus nephritis is often detected as a nephrotic syndrome after 3-6 months. In the subacute course, undulation is distinct with the involvement of various organs and systems in the pathological process and characteristic polysyndromicity. The chronic course of the disease for a long time is characterized by recurrences of polyarthritis and (or) polyserositis, syndromes of discoid lupus, Raynaud; only at the 5-10th year characteristic polysyndromicity gradually develops. In accordance with the clinical and laboratory characteristics, three degrees of process activity are distinguished; high (III degree), moderate (II degree) and minimal (I degree).

Treatment. Patients need continuous long-term complex treatment. The best results with the development of stable clinical remission - with early treatment. In chronic and subacute course and I degree of activity, non-steroidal anti-inflammatory drugs and aminoquinoline derivatives are indicated. The former are recommended for articular syndrome. The selection of the drug is important, taking into account its individual efficacy and tolerability: voltaren (ortofen) 50 mg 2-3 times a day, indomethacin 25-50 mg 2-3 times a day, brufen 400 mg 3 times a day, hingamin (chloroquine, delagil) at 0.25-0.5 g / day for 10-14 days, and then at 0.25 r / day for several months. With the development of diffuse lupus nephritis, plaquenil is successfully used at a dose of 0.2 g 4-5 times a day for a long time under control of the dynamics of the urinary syndrome.

In acute course from the very beginning, and in subacute and chronic course at III, II degree of activity of the pathological process, glucocorticosteroids are indicated. The initial dose of these drugs should be sufficient to reliably suppress the activity of the pathological process. Prednisolone at a dose of 40-60 mg / day is prescribed for acute and subacute course with III degree of activity and the presence of nephrotic syndrome or meningoencephalitis. In the same variants of the course with the II degree of activity, as well as in the chronic course with the III and II degree of activity, the suppressive dose should be 30-40 mg, and with the I degree of activity - 15-20 mg / day. Treatment with prednisolone in an overwhelming dose is carried out until a pronounced clinical effect occurs (according to a decrease in clinical and laboratory activity indicators). Upon reaching the effect, the dose of prednisolone is slowly reduced, focusing on the proposed scheme (Table 7).

Table 7 Approximate scheme reducing the dose of prednisolone when a therapeutic effect is achieved

Dose							A week
prednisolone, mg	1st	3rd	4th	5th	6th	7th	8th
75	70	60	50
50	47,5	45	42,5	42,5	40
40	37,5	35	35	32,5	32,5	30	30
30	27,5	25	25	22,5	22,5	29	20*
* Then very slowly - half a tablet (2.5 mg) in 1-3 months (according to indicators general condition and laboratory data).

One of critical tasks and the key to the effectiveness of therapy is the selection of the smallest dose that allows you to maintain clinical and laboratory remission. Prednisone at a maintenance dose of 5-10 mg/day is prescribed for several years.

For decreasing side effect it is recommended to combine this therapy with potassium preparations, anabolic steroids, diuretics and antihypertensive drugs, tranquilizers, antiulcer measures. The most serious complications: steroid ulcer, septic infections, tuberculosis, candidiasis, psychosis.

With an aggressive course of the disease, a high titer of autoantibodies, immune complexes, plasmapheresis is successfully used.

With the ineffectiveness of glucocorticoids, immunosuppressants (alkylating series or azathioprine) are prescribed. Indications for the administration of cytotoxic drugs (usually in combination with moderate doses of corticosteroids) are as follows:

1) 1 degree of activity in adolescents and menopause;

2) nephrotic and nephritic syndromes;

3) the need to quickly reduce the suppressive dose of prednisolone due to the severity side effects(rapid and significant weight gain, excessive arterial hypertension, steroid diabetes, severe osteoporosis with signs of spondylopathy);

4) the need to reduce the maintenance dose of prednisolone if it exceeds 15-20 mg / day.

The most commonly used are azathioprine (Imuran) and cyclophosphamide (cyclophosphamide) at a dose of 1-3 mg/kg (100-200 mg/day) in combination with 30 mg of prednisolone. At this dose, the drug is prescribed for 2-2.5 months, usually in a hospital, then a maintenance dose (50-100 mg per day) is recommended, which is given for several months and even 1-2 years or more.

To ensure the safety of treatment, careful monitoring of blood counts is required to prevent pancytopenia; connection must be avoided. infectious complications, dyspeptic complications; when taking cyclophosphamide, the risk of developing hemorrhagic cystitis can be reduced by prescribing plentiful drink(2 liters of fluid or more per day).

Since patients need many years of treatment after discharge from the hospital, they should be under the supervision of a general practitioner or rheumatologist in the clinic. In order to improve the tolerability of long-term corticosteroid therapy in outpatient settings, delagil 0.25 g / day and B vitamins are recommended, ascorbic acid in the form of a spring-autumn course. Patients are shown treatment in sanatoriums of the local type (cardiological, rheumatological). Climatobalneological, physiotherapeutic treatment is contraindicated, since ultraviolet irradiation, insolation and hydrotherapy can exacerbate the disease.

scleroderma systemic- a chronic systemic disease of connective tissue and small vessels with widespread fibro-sclerotic changes in the skin and stroma of internal organs and symptoms of obliterating endarteritis in the form of systemic Raynaud's syndrome.

Etiology unknown. It is provoked by cooling, trauma, infection, vaccination, etc.

In the pathogenesis of the leading role is the violation of collagen metabolism associated with functional hyperactivity of fibroblasts and smooth muscle cells of the vascular wall. Not less than an important factor pathogenesis is a violation of microcirculation due to damage to the vascular wall and a change in the intravascular aggregate properties of the blood. In a sense, systemic scleroderma is a typical collagen disease associated with excessive collagen formation (and fibrosis) of functionally defective fibroblasts and other collagen-forming cells. Family genetic predisposition matters. Women get sick 3 times more often than men.

Symptoms, course. Usually the disease begins with Raynaud's syndrome (vasomotor disorders), trophic disorders or persistent arthralgia, weight loss, fever, asthenia. Starting with any one symptom, systemic scleroderma gradually or rather quickly acquires the features of a multisyndromic disease.

Skin lesion is a pathognomonic sign of the disease. This is a widespread dense edema, in the future - thickening and atrophy of the skin. Biggest changes undergo the skin of the face and limbs; often the skin of the entire body is dense. At the same time, focal or widespread pigmentation develops with areas of depigmentation, telangiectasia. Ulcerations and pustules on the fingertips are characteristic, which do not heal for a long time and are extremely painful, deformation of the nails, hair loss up to baldness and other trophic disorders.

Often develops fibrosing interstitial myositis. muscle syndrome accompanied by myalgia, progressive induration, then muscle atrophy, decreased muscle strength. Only in rare cases there is acute polymyositis with pain, swelling of the muscle, etc. Fibrosing changes in the muscles are accompanied by fibrosis of the tendons, which leads to muscle-tendon contractures - one of the reasons for the relatively early disability of patients. Joint damage is mainly associated with pathological processes in periarticular tissues (skin, tendons, articular bags, muscles). Arthralgia is observed in 80-90% of patients, often accompanied by severe deformity of the joints due to proliferative changes in the periarticular tissues; X-ray examination does not reveal significant destruction. An important diagnostic feature is osteolysis of the terminal, and in severe cases, middle phalanges of the fingers, less often the legs. The deposition of calcium salts in subcutaneous tissue localized mainly in the area of the fingers and periarticular tissues, expressed in the form of painful uneven formations, sometimes spontaneously opening with rejection of crumbly calcareous masses.

Damage to the cardiovascular system observed in almost all patients: the myocardium and endocardium are affected, rarely the pericardium. Scleroderma cardiosclerosis is clinically characterized by pain in the region of the heart, shortness of breath, extrasystole, muffled tones and systolic murmur at the top, the expansion of the heart to the left. An X-ray examination shows a weakening of the pulsation and smoothness of the contours of the heart, with X-ray kymography - silent zones in areas of large-focal cardiosclerosis; and in the most severe cases, an aneurysm of the heart is formed in connection with the replacement muscle tissue fibrous. On the ECG, a decrease in voltage, conduction disturbances up to atrioventricular blockade are usually observed; heart attack-like ECG occurs with the development of massive foci of fibrosis in the myocardium. If the process is localized in the endocardium, the development of scleroderma heart disease and damage to the parietal endocardium are possible. Usually suffers mitral valve. Scleroderma heart disease is characterized by a benign course. Heart failure rarely develops, mainly with widespread damage to the heart muscle or all three of its membranes.

Damage to small arteries arterioles cause such peripheral symptoms of scleroderma as Raynaud's syndrome, gangrene of the fingers. Damage to the vessels of internal organs leads to severe visceral pathology - hemorrhages, ischemic and even necrotic changes With clinical picture severe visceritis (decay of lung tissue, "true scleroderma kidney", etc.). Vascular pathology determines the speed of the process, its severity and often the outcome of the disease. At the same time, it is possible to damage large vessels with a clinical picture of thromboangiitis obliterans; ischemic phenomena develop, and often gangrene of the toes, migrating thrombophlebitis with strophic ulcers in the feet and legs, etc.

Lung injury in the form of diffuse or focal pneumofibrosis, mainly in the basal parts of the lungs, usually accompanied by emphysema and bronchiectasis, and often adhesive pleurisy. Shortness of breath, difficulty deep breath, hard breathing, wheezing during auscultation of the lungs, a boxy shade of percussion sound, a decrease in vital capacity to 40-60% of the due, bilateral amplification and deformation of the lung pattern, sometimes with a fine-meshed structure (" honeycomb»); at X-ray examination - signs that usually characterize scleroderma pneumofibrosis. Kidney damage is more often manifested by focal nephritis, but diffuse glomerulonephritis with hypertension and renal failure is possible. With a rapidly progressive course systemic scleroderma often develops "true scleroderma kidney" due to damage to the vessels of the kidney, leading to focal necrosis of the cortex and renal failure. The defeat of the esophagus, manifested by dysphagia, expansion, weakening of peristalsis and rigidity of the walls with a slowdown in the passage of barium during X-ray examination, is observed very often and is of great diagnostic value. Often in the lower part of the rigid esophagus are formed peptic ulcers. In connection with the defeat of the vessels, the development of ulcers, hemorrhages, ischemic necrosis and bleeding in the digestive tract is possible.

Damage to the nervous system manifested by polyneuritis, vegetative instability (impaired sweating, thermoregulation, vasomotor skin reactions), emotional lability, irritability, tearfulness and suspiciousness, insomnia. Only in rare cases does a picture of encephalitis or psychosis occur. Possible symptoms of sclerosis of cerebral vessels due to their scleroderma lesion, even in individuals young age. There is a lesion of reticuloendothelial (popiadenia, and in some patients hepatosplenomegaly) and endocrine (pluriglandular insufficiency or pathology of one or another gland internal secretion) systems.

More often there is a chronic course, the disease lasts for decades with minimal activity process and the gradual spread of lesions to different internal organs, whose function is not disturbed for a long time. Such patients suffer mainly from damage to the skin, joints and trophic disorders. Within the framework of chronic systemic scleroderma, CRST syndrome (calcification, Raynaud's syndrome, sclerodactyly and telangiectasia) is distinguished, characterized by a long benign course with an extremely slow development of visceral pathology. In a subacute course, the disease begins with sartrapgia, weight loss, visceral pathology rapidly increases, and the disease acquires a steadily progressive course with the spread of the pathological process to many organs and systems. Death usually occurs 1-2 years after the onset of the disease.

Laboratory data are not representative. Moderate normo- or hypochromic anemia, moderate leukocytosis and eosinophilia, transient thrombocytopenia are usually observed. ESR is normal or moderately elevated in chronic course and significantly increased (up to 50-60 mm/h) in subacute.

Treatment: the use of anti-inflammatory and restorative agents, the restoration of lost functions of the musculoskeletal system.

Active anti-inflammatory therapy with corticosteroids is indicated mainly in the subacute course or during periods of pronounced process activity in the chronic course. Prednisolone 20-30 mg is given for 1-1.5 months until a pronounced therapeutic effect is achieved, then it is very slowly reduced, the maintenance dose (5-10 mg of prednisolone) is used for a long time, until a lasting effect is obtained. Non-steroidal anti-inflammatory drugs can be recommended during the period of reducing the doses of hormonal drugs. D-penicillamine is prescribed 150 mg 3-4 times a day with a gradual increase up to 6 times a day (900 mg) for a long time, at least a year; especially indicated in the rapid progression of the disease; most serious complication- nephrotic syndrome requiring immediate discontinuation of the drug; dyspeptic disorders decrease with temporary discontinuation of the drug, changes in taste can be corrected by the appointment of vitamin B6. Aminoquinoline preparations are indicated for all variants of the course. Delagil (0.25 g 1 time per day) or Plaquenil (0.2 g 2 times a day) can be prescribed for a long time, for years, especially with the leading articular syndrome.

In recent years, calcium channel blockers have been widely used - Corinfar (nifedipine) at 30-80 mg / day, for months with good tolerance. With "true scleroderma kidney" - plasmapheresis, long-term use captopril 400 mg per day (up to 1 year or more).

In chronic course, lidase (hyaluronidase) is recommended, under the influence of which stiffness decreases and mobility in the joints increases, mainly due to softening of the skin and underlying tissues. Lidase is administered every other day at 64 AU in a 0.5% solution of novocaine s / c (12 injections per course). After 1-2 months, the course of treatment with lidase can be repeated (only 4-6 courses per year). With a pronounced angiospastic component (Raynaud's syndrome), repeated courses of angiotrophin are shown (1 ml s / c, for a course of 30 injections), kallikrein depot, andecalin (1 ml intramuscularly, for a course of 30 injections).

In all variants of the course of the disease, active vitamin therapy, ATP is recommended. In a chronic course, balneotherapy is indicated (coniferous, radon and hydrogen sulfide baths), paraffin and mud applications, hyaluronidase electrophoresis, applications with 30-50% dimethyl sulfoxide solution (20-30 sessions) on the affected limbs. Physiotherapy exercises and massage are important. In subacute course, morning hygienic exercises and an active position in bed are recommended, in chronic cases, persistent and prolonged use of therapeutic exercises in combination with massage and various labor processes (molding from warm paraffin, weaving, sawing, etc.).