Studies of biochemical parameters of blood in lupus erythematosus. Criteria for the diagnosis of systemic lupus erythematosus

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  • Diagnostics
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  • Treatment
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  • Diagnosis of lupus erythematosus

    General principles for diagnosing a disease

    Diagnosis of systemic lupus erythematosus exhibited on the basis of special developed diagnostic criteria proposed by the American Association of Rheumatologists or Russian scientist Nasonova. Further, after the diagnosis is made on the basis of diagnostic criteria, additional examinations are performed - laboratory and instrumental, which confirm the correctness of the diagnosis and allow assessing the degree of activity pathological process and identify affected organs.

    Currently, the most commonly used diagnostic criteria American Association rheumatologists, not Nasonova. But we will give both schemes of diagnostic criteria, since in a number of cases, domestic doctors use Nasonova's criteria to diagnose lupus.

    Diagnostic criteria of the American Rheumatology Association the following:

    • Rashes in the cheekbones on the face (there are red elements of the rash that are flat or slightly rising above the skin surface, extending to the nasolabial folds);
    • Discoid rashes (plaques raised above the skin surface with "black dots" in the pores, peeling and atrophic scars);
    • Photosensitivity (the appearance of rashes on the skin after exposure to the sun);
    • Ulcers on the mucous membrane of the oral cavity (painless ulcerative defects localized on the mucous membrane of the mouth or nasopharynx);
    • Arthritis (damage to two or more small joints, characterized by pain, swelling and swelling);
    • Polyserositis (pleurisy, pericarditis, or non-infectious peritonitis, present or past);
    • Kidney damage (the constant presence of protein in the urine in an amount of more than 0.5 g per day, as well as the constant presence of erythrocytes and cylinders in the urine (erythrocyte, hemoglobin, granular, mixed));
    • Neurological disorders: seizures or psychosis (delusions, hallucinations) not due to medication, uremia, ketoacidosis, or electrolyte imbalance;
    • Hematological disorders (hemolytic anemia, leukopenia with the number of leukocytes in the blood less than 1 * 10 9, lymphopenia with the number of lymphocytes in the blood less than 1.5 * 10 9, thrombocytopenia with the number of platelets less than 100 * 10 9);
    • Immunological disorders (antibodies to double-stranded DNA in an increased titer, the presence of antibodies to the Sm antigen, a positive LE test, a false positive Wasserman reaction to syphilis for six months, the presence of an antilupus coagulant);
    • An increase in the titer of ANA (antinuclear antibodies) in the blood.
    If a person has any four of the above signs, then he definitely has systemic lupus erythematosus. In this case, the diagnosis is considered accurate and confirmed. If a person has only three of any of the above, then the diagnosis of lupus erythematosus is considered only probable, and data from laboratory tests and instrumental examinations are needed to confirm it.

    Criteria for lupus erythematosus Nasonova include major and minor diagnostic criteria, which are shown in the table below:

    Great diagnostic criteria Minor diagnostic criteria
    "Butterfly on the Face"Body temperature above 37.5 o C, lasting longer than 7 days
    ArthritisCauseless weight loss of 5 or more kg in a short time and malnutrition of tissues
    Lupus pneumonitiscapillaries on the fingers
    LE cells in the blood (less than 5 per 1000 leukocytes - single, 5 - 10 per 1000 leukocytes - moderate amount, and more than 10 per 1000 leukocytes - a large number)Rashes on the skin like urticaria or rash
    ANF ​​in high creditsPolyserositis (pleurisy and carditis)
    Werlhof syndromeLymphadenopathy (increased lymphatic ducts and nodes)
    Coombs-positive hemolytic anemiaHepatosplenomegaly (enlargement of the liver and spleen)
    Lupus JadeMyocarditis
    Hematoxylin bodies in pieces of tissues of various organs taken during a biopsyCNS lesion
    Characteristic pathomorphological picture in the removed spleen ("bulbous sclerosis"), in skin samples (vasculitis, immunofluorescent glow of immunoglobulins on the basement membrane) and kidneys (glomerular capillary fibrinoid, hyaline thrombi, "wire loops")Polyneuritis
    Polymyositis and polymyalgia (inflammation and muscle pain)
    Polyarthralgia (joint pain)
    Raynaud's syndrome
    ESR acceleration over 200 mm/h
    Decrease in the number of leukocytes in the blood less than 4 * 10 9 / l
    Anemia (hemoglobin level below 100 mg/ml)
    Reducing the number of platelets below 100 * 10 9 / l
    Increase in the amount of globulin proteins over 22%
    ANF ​​in low credits
    Free LE bodies
    Positive Wassermann test with confirmed absence of syphilis


    The diagnosis of lupus erythematosus is considered accurate and confirmed when any of the three major diagnostic criteria are combined, and one of them must be either "butterfly" or LE cells in in large numbers, and the other two are any of the above. If a person has only minor diagnostic signs or they are combined with arthritis, then the diagnosis of lupus erythematosus is considered only probable. In this case, to confirm it, data from laboratory tests and additional instrumental examinations are required.

    The above criteria of Nason and the American Association of Rheumatologists are the main ones in the diagnosis of lupus erythematosus. This means that the diagnosis of lupus erythematosus is made only on their basis. And any laboratory tests and instrumental methods of examination are only additional, allowing to assess the degree of activity of the process, the number of affected organs and the general condition of the human body. Based on laboratory tests and instrumental methods examinations, the diagnosis of lupus erythematosus is not made.

    Currently, ECG, EchoCG, MRI, chest X-ray, ultrasound, etc. can be used as instrumental diagnostic methods for lupus erythematosus. All these methods make it possible to assess the degree and nature of damage in various organs.

    Blood (test) for lupus erythematosus

    Among the laboratory tests to assess the degree of intensity of the process in lupus erythematosus, the following are used:
    • Antinuclear factors (ANF) - with lupus erythematosus are found in the blood in high titers not higher than 1: 1000;
    • Antibodies to double-stranded DNA (anti-dsDNA-AT) - with lupus erythematosus are found in the blood in 90 - 98% of patients, and are normally absent;
    • Antibodies to histone proteins - with lupus erythematosus are found in the blood, are normally absent;
    • Antibodies to the Sm antigen - with lupus erythematosus are found in the blood, but are normally absent;
    • Antibodies to Ro / SS-A - in lupus erythematosus are found in the blood if there is lymphopenia, thrombocytopenia, photosensitivity, pulmonary fibrosis, or Sjögren's syndrome;
    • Antibodies to La / SS-B - in lupus erythematosus are found in the blood under the same conditions as antibodies to Ro / SS-A;
    • Complement level - in lupus erythematosus, the level of complement proteins in the blood is reduced;
    • The presence of LE cells - in lupus erythematosus, they are found in the blood in 80 - 90% of patients, and are normally absent;
    • Antibodies to phospholipids (lupus anticoagulant, antibodies to cardiolipin, positive Wassermann test with confirmed absence of syphilis);
    • Antibodies to coagulation factors VIII, IX and XII (normally absent);
    • Increase in ESR more than 20 mm/hour;
    • Leukopenia (decrease in the level of leukocytes in the blood less than 4 * 10 9 / l);
    • Thrombocytopenia (decrease in the level of platelets in the blood less than 100 * 10 9 / l);
    • Lymphopenia (decrease in the level of lymphocytes in the blood is less than 1.5 * 10 9 / l);
    • Elevated blood concentrations of seromucoid, sialic acids, fibrin, haptoglobin, C-reactive protein of circulating immune complexes and immunoglobulins.
    At the same time, tests for the presence of lupus anticoagulant, antibodies to phospholipids, antibodies to the Sm factor, antibodies to histone proteins, antibodies to La / SS-B, antibodies to Ro / SS-A, LE cells, antibodies to double stranded DNA and antinuclear factors.

    Diagnosis of lupus erythematosus, tests. How to distinguish lupus erythematosus from psoriasis, eczema, scleroderma, lichen and urticaria (recommendations from a dermatologist) - video

    Treatment of systemic lupus erythematosus

    General principles of therapy

    Since the exact causes of lupus erythematosus are unknown, there are no therapies that can completely cure this disease. As a result, only pathogenetic therapy, the purpose of which is to suppress the inflammatory process, prevent relapses and achieve stable remission. In other words, the treatment of lupus erythematosus is to slow down the progression of the disease as much as possible, lengthen the periods of remission and improve the quality of human life.

    The main drugs in the treatment of lupus erythematosus are glucocorticosteroid hormones.(Prednisolone, Dexamethasone, etc.), which are used constantly, but depending on the activity of the pathological process and the severity of the general condition of the person, they change their dosage. The main glucocorticoid in the treatment of lupus is Prednisolone. It is this drug that is the drug of choice, and it is for him that the exact dosages for various clinical options and activity of the pathological process of the disease. Dosages for all other glucocorticoids are calculated based on prednisolone dosages. The list below shows dosages of other glucocorticoids equivalent to 5 mg of prednisolone:

    • Betamethasone - 0.60 mg;
    • Hydrocortisone - 20 mg;
    • Dexamethasone - 0.75 mg;
    • Deflazacort - 6 mg;
    • Cortisone - 25 mg;
    • Methylprednisolone - 4 mg;
    • Paramethasone - 2 mg;
    • Prednisone - 5 mg;
    • Triamcinolone - 4 mg;
    • Flurprednisolone - 1.5 mg.
    Glucocorticoids are constantly taken, changing the dosage depending on the activity of the pathological process and the general condition of the person. During periods of exacerbations, hormones are taken at a therapeutic dosage for 4 to 8 weeks, after which, upon reaching remission, they continue to take them at a lower maintenance dosage. In a maintenance dosage, Prednisolone is taken throughout life during periods of remission, and during exacerbations, the dosage is increased to therapeutic.

    So, at the first degree of activity pathological process Prednisolone is used in therapeutic dosages of 0.3 - 0.5 mg per 1 kg of body weight per day, at the second degree of activity- 0.7 - 1.0 mg per 1 kg of weight per day, and at the third degree- 1 - 1.5 mg per 1 kg of body weight per day. In the indicated doses, Prednisolone is used for 4 to 8 weeks, and then the dosage of the drug is reduced, but it is never completely canceled. The dosage is first reduced by 5 mg per week, then by 2.5 mg per week, after a while, by 2.5 mg in 2 to 4 weeks. In total, the dosage is reduced so that 6-9 months after the start of taking Prednisolone, its dose becomes maintenance, equal to 12.5-15 mg per day.

    With a lupus crisis, capturing several organs, glucocorticoids are administered intravenously for 3 to 5 days, after which they switch to taking drugs in tablets.

    Since glucocorticoids are the main means of treating lupus, they are prescribed and used without fail, and all other drugs are used additionally, selecting them depending on the severity of clinical symptoms and on the affected organ.

    So, with a high degree of activity of lupus erythematosus, with lupus crises, with severe lupus nephritis, with severe damage to the central nervous system, with frequent relapses and instability of remission, in addition to glucocorticoids, cytostatic immunosuppressants are used (Cyclophosphamide, Azathioprine, Cyclosporine, Methotrexate, etc.).

    With severe and widespread lesions of the skin Azathioprine is used at a dosage of 2 mg per 1 kg of body weight per day for 2 months, after which the dose is reduced to maintenance: 0.5-1 mg per 1 kg of body weight per day. Azathioprine at a maintenance dosage is taken for several years.

    For severe lupus nephritis and pancytopenia(decrease in the total number of platelets, erythrocytes and leukocytes in the blood) use Cyclosporine at a dosage of 3-5 mg per 1 kg of body weight.

    With proliferative and membranous lupus nephritis, with severe damage to the central nervous system Cyclophosphamide is used, which is administered intravenously at a dosage of 0.5 - 1 g per m 2 of body surface once a month for six months. Then, for two years, the drug continues to be administered at the same dosage, but once every three months. Cyclophosphamide ensures the survival of patients suffering from lupus nephritis and helps control clinical symptoms that are not affected by glucocorticoids (CNS damage, pulmonary hemorrhage, pulmonary fibrosis, systemic vasculitis).

    If lupus erythematosus does not respond to glucocorticoid therapy, then Methotrexate, Azathioprine or Cyclosporine are used instead.

    With low activity of the pathological process with lesions skin and joints in the treatment of lupus erythematosus, aminoquinoline drugs are used (Chloroquine, Hydroxychloroquine, Plaquenil, Delagil). In the first 3-4 months, drugs are used at 400 mg per day, and then at 200 mg per day.

    With lupus nephritis and the presence of antiphospholipid bodies in the blood(antibodies to cardiolipin, lupus anticoagulant) drugs of the group of anticoagulants and antiaggregants (Aspirin, Curantil, etc.) are used. Mainly use acetylsalicylic acid in small doses - 75 mg per day for a long time.

    Drugs of the group of non-steroidal anti-inflammatory drugs (NSAIDs), such as Ibuprofen, Nimesulide, Diclofenac, etc., are used as drugs to relieve pain and relieve inflammation in arthritis, bursitis, myalgia, myositis, moderate serositis and fever.

    Apart from medicines, for the treatment of lupus erythematosus, methods of plasmapheresis, hemosorption and cryoplasmosorption are used, which allow the removal of antibodies and inflammatory products from the blood, which significantly improves the condition of patients, reduces the degree of activity of the pathological process and reduces the rate of progression of the pathology. However, these methods are only auxiliary, and therefore can only be used in combination with taking medications, and not instead of them.

    For the treatment of skin manifestations of lupus, it is necessary to externally use sunscreens with UVA and UVB filters and ointments with topical steroids (Ftorcinolone, Betamethasone, Prednisolone, Mometasone, Clobetasol, etc.).

    Currently, in addition to these methods, drugs of the group of tumor necrosis factor blockers (Infliximab, Adalimumab, Etanercept) are used in the treatment of lupus. However, these drugs are used exclusively as a trial, experimental treatment, since they are not currently recommended by the Ministry of Health. But the results obtained allow us to consider tumor necrosis factor blockers as promising drugs, since the effectiveness of their use is higher than that of glucocorticoids and immunosuppressants.

    In addition to the described drugs used directly for the treatment of lupus erythematosus, this disease shows the intake of vitamins, potassium compounds, diuretics and antihypertensive drugs, tranquilizers, antiulcers and other drugs that reduce the severity of clinical symptoms from various organs, as well as restoring normal exchange substances. With lupus erythematosus, you can and should additionally use any drugs that improve general well-being person.

    Drugs for lupus erythematosus

    Currently, the following groups of drugs are used to treat lupus erythematosus:
    • Glucocorticosteroids (Prednisolone, Methylprednisolone, Betamethasone, Dexamethasone, Hydrocortisone, Cortisone, Deflazacort, Paramethasone, Triamcinolone, Flurprednisolone);
    • Cytostatic immunosuppressants (Azathioprine, Methotrexate, Cyclophosphamide, Cyclosporine);
    • Antimalarial drugs - aminoquinoline derivatives (Chloroquine, Hydroxychloroquine, Plaquenil, Delagil, etc.);
    • Alpha TNF blockers (Infliximab, Adalimumab, Etanercept);
    • Non-steroidal anti-inflammatory drugs (Diclofenac, Nimesulide,

    Systemic lupus erythematosus- a chronic systemic disease, with the most pronounced manifestations on the skin; the etiology of lupus erythematosus is not known, but its pathogenesis is associated with a violation of autoimmune processes, resulting in the production of antibodies to healthy cells organism. The disease is more susceptible to middle-aged women. The incidence of lupus erythematosus is not high - 2-3 cases per thousand people of the population. Treatment and diagnosis of systemic lupus erythematosus is carried out jointly by a rheumatologist and a dermatologist. The diagnosis of SLE is based on typical clinical signs, laboratory test results.

    General information

    Systemic lupus erythematosus- a chronic systemic disease, with the most pronounced manifestations on the skin; The etiology of lupus erythematosus is not known, but its pathogenesis is associated with a violation of autoimmune processes, resulting in the production of antibodies to healthy cells of the body. The disease is more susceptible to middle-aged women. The incidence of lupus erythematosus is not high - 2-3 cases per thousand people of the population.

    Development and suspected causes of systemic lupus erythematosus

    The exact etiology of lupus erythematosus has not been established, but antibodies to the Epstein-Barr virus were found in most patients, which confirms the possible viral nature diseases. Features of the body, due to which autoantibodies are produced, are also observed in almost all patients.

    The hormonal nature of lupus erythematosus has not been confirmed, but hormonal disorders worsen the course of the disease, although they cannot provoke its occurrence. Women diagnosed with lupus erythematosus are not recommended to take oral contraceptives. In people with a genetic predisposition and in identical twins, the incidence of lupus erythematosus is higher than in other groups.

    The pathogenesis of systemic lupus erythematosus is based on impaired immunoregulation, when protein components of the cell, primarily DNA, act as autoantigens, and as a result of adhesion, even those cells that were originally free of immune complexes become targets.

    Clinical picture of systemic lupus erythematosus

    With lupus erythematosus, connective tissue, skin and epithelium are affected. Important diagnostic sign is a symmetrical lesion of large joints, and if joint deformity occurs, then due to the involvement of ligaments and tendons, and not due to erosive lesions. Myalgia, pleurisy, pneumonitis are observed.

    But the most striking symptoms of lupus erythematosus are noted on the skin, and it is precisely for these manifestations that the diagnosis is made in the first place.

    On initial stages disease lupus erythematosus is characterized by a continuous course with periodic remissions, but almost always goes into a systemic form. More often there is erythematous dermatitis on the face like a butterfly - erythema on the cheeks, cheekbones and always on the back of the nose. There is hypersensitivity to solar radiation- photodermatosis usually round shape, are plural. In lupus erythematosus, a feature of photodermatosis is the presence of a hyperemic corolla, an area of ​​atrophy in the center, and depigmentation of the affected area. Pityriasis scales, which cover the surface of erythema, are tightly soldered to the skin and attempts to separate them are very painful. At the stage of atrophy of the affected skin, the formation of a smooth, delicate alabaster-white surface is observed, which gradually replaces the erythematous areas, starting from the middle and moving to the periphery.

    In some patients with lupus erythematosus, the lesions extend to the scalp, causing total or partial alopecia. If the lesions affect the red border of the lips and the mucous membrane of the mouth, then the lesions are bluish-red dense plaques, sometimes with pityriasis scales on top, their contours have clear boundaries, the plaques are prone to ulceration and cause pain during eating.

    Lupus erythematosus has a seasonal course, and in the autumn-summer periods, the skin condition deteriorates sharply due to more intense exposure to solar radiation.

    In the subacute course of lupus erythematosus, psoriasis-like foci are observed throughout the body, telangiectasias are pronounced, a reticular livedio (tree-like pattern) appears on the skin of the lower extremities. Generalized or alopecia areata, urticaria and pruritus are observed in all patients with systemic lupus erythematosus.

    In all organs where there is connective tissue, pathological changes begin over time. With lupus erythematosus, all membranes of the heart, renal pelvis, gastrointestinal tract and central nervous system are affected.

    If, in addition to skin manifestations, patients suffer from recurrent headaches, joint pains without connection with injuries and weather conditions, there are violations of the work of the heart and kidneys, then already on the basis of the survey, one can assume about deeper and systemic disorders and examine the patient for the presence of lupus erythematosus. Abrupt change moods from a euphoric state to a state of aggression is also a characteristic manifestation of lupus erythematosus.

    In elderly patients with lupus erythematosus skin manifestations, renal and arthralgic syndromes are less pronounced, but Sjögren's syndrome is more often observed - this is an autoimmune lesion connective tissue manifested by hyposecretion salivary glands, dryness and pain in the eyes, photophobia.

    Children with neonatal lupus erythematosus, born to sick mothers, have an erythematous rash and anemia already in infancy, so a differential diagnosis should be made with atopic dermatitis.

    Diagnosis of systemic lupus erythematosus

    If systemic lupus erythematosus is suspected, the patient is referred for a consultation with a rheumatologist and a dermatologist. Lupus erythematosus is diagnosed by the presence of manifestations in each symptomatic group. Criteria for diagnosis from the skin: butterfly-shaped erythema, photodermatitis, discoid rash; on the part of the joints: symmetrical joint damage, arthralgia, "pearl bracelet" syndrome on the wrists due to deformity ligamentous apparatus; on the part of the internal organs: serositis of various localization, persistent proteinuria and cylindruria in the analysis of urine; from the side of the central nervous system: convulsions, chorea, psychosis and mood changes; from the function of hematopoiesis, lupus erythematosus is manifested by leukopenia, thrombocytopenia, lymphopenia.

    The Wasserman reaction can be false positive, like other serological studies, which sometimes leads to the appointment of inadequate treatment. With the development of pneumonia, an x-ray of the lungs is performed, if pleurisy is suspected -

    Patients with lupus erythematosus should avoid direct sunlight, wear clothing that covers the entire body, and apply creams with a high protective UV filter to exposed areas. Corticosteroid ointments are applied to the affected areas of the skin, since the use of non-hormonal drugs has no effect. Treatment must be carried out intermittently so that hormone-mediated dermatitis does not develop.

    In uncomplicated forms of lupus erythematosus to eliminate pain non-steroidal anti-inflammatory drugs are prescribed in muscles and joints, but aspirin should be taken with caution, as it slows down the blood clotting process. It is obligatory to take glucocorticosteroids, while the doses of drugs are selected in such a way as to minimize side effects to protect internal organs from damage.

    The method when a patient is taken stem cells, and then immunosuppressive therapy is carried out, after which for recovery immune system reintroduced stem cells, effective even in severe and hopeless forms of lupus erythematosus. With such therapy, autoimmune aggression in most cases stops, and the patient's condition with lupus erythematosus improves.

    Healthy lifestyle, avoidance of alcohol and smoking, adequate exercise stress, balanced diet and psychological comfort allow patients with lupus erythematosus to control their condition and prevent disability.

    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). Proven protective action regarding SLE of male sex hormones (androgens). 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 sunbeams(photosensitization), 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 serious condition as a 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: main symptom - dull pain in the chest area. 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: cardiac arrhythmias, conduction disturbances 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 develop in young people patients with SLE.
    • Damage to the inner lining of blood vessels (endothelium) increases the risk of atherosclerosis. Defeat peripheral vessels manifests itself:
      • 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, most common symptoms with 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, increased The lymph nodes, 90% of patients are diagnosed with enlarged spleen (splenomegaly).

    Diagnosis of SLE


    The diagnosis of SLE is based on data clinical manifestations diseases, as well as on the data of 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.

    1. Arthritis
    Characteristic: without erosion, peripheral, manifested by pain, swelling, accumulation of insignificant fluid in the joint cavity
    1. discoid rashes
    Red, oval, round or annular in shape, plaques with uneven contours on their surface there are scales, dilated capillaries nearby, the scales are separated with difficulty. Untreated lesions leave scars.
    1. Mucosal lesions
    The oral mucosa or nasopharyngeal mucosa is affected in the form of ulcerations. Usually painless.
    1. photosensitization
    Increased sensitivity to sunlight. As a result of exposure to sunlight, a rash appears on the skin.
    1. Rash on back of nose and cheeks
    Specific rash in the form of a butterfly
    1. Kidney damage
    Permanent loss of protein in the urine 0.5 g/day, excretion of cellular casts
    1. 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
    1. CNS lesion
    Convulsions, Psychosis - in the absence of drugs that can provoke them or metabolic disorders (uremia, etc.)
    1. 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
    1. Changes in the immune system
    • Altered amount of anti-DNA antibodies
    • Presence of cardiolipin antibodies
    • Antinuclear antibodies anti-Sm
    1. 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) Need 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 logical thinking, orientation in space is disturbed, memory, intelligence, concentration, incoherent speech, insomnia or drowsiness are reduced. 8
    Eye disorders Inflammation optic nerve except for 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 skeletal muscle) 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 Damage inflammatory nature 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

    1. 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
    2. Anti DNA– antibodies to DNA, determined in 50% of patients, the level of these antibodies reflects the activity of the disease
    3. Anti-sm- specific antibodies to the Smith antigen, which is part of short RNA, are detected in 30-40% of cases
    4. 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
    5. Anticardiolipin - antibodies to mitochondrial membranes (energy station of cells)
    6. 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 progress in modern treatment systemic lupus erythematosus, this task remains very difficult. Treatment aimed at eliminating main reason 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. Medical treatment
    1. Glucocorticosteroids the most effective drugs in the treatment of SLE.
    Long-term glucocorticosteroid therapy in patients with SLE has been shown to maintain good quality life and increase 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 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, lightning fast lupus nephritis, high immunological activity, damage to the nervous system.

    • 1000 mg methylprednisolone and 1000 mg cyclophosphamide on the first day
    1. Cytostatics: cyclophosphamide (cyclophosphamide), azathioprine, methotrexate, are used in complex treatment 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
    1. Anti-inflammatory drugs
    They are used at high temperature, with damage to the joints, and serositis.
    • Naklofen, nimesil, aertal, catafast, etc.
    1. Aminoquinoline drugs
    They have anti-inflammatory and immunosuppressive effects, are used in hypersensitivity to sunlight and skin lesions.
    • delagil, plaquenil, etc.
    1. Biologicals are promising method treatment of SLE
    These drugs have far fewer side effects than hormonal preparations. They have a narrowly focused effect on the mechanisms of development immune diseases. Effective but costly.
    • Anti CD 20 - Rituximab
    • Tumor necrosis factor alpha - Remicade, Gumira, Embrel
    1. Other drugs
    • Anticoagulants (heparin, warfarin, etc.)
    • Antiplatelet agents (aspirin, clopidogrel, etc.)
    • Diuretics (furosemide, hydrochlorothiazide, etc.)
    • Calcium and potassium preparations
    1. 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- most favorable course 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 , it is very rare to prescribe short courses hormone therapy(Prednisolone). Diagnosis is set by the method of exclusion: if the symptoms of lupus erythematosus began immediately after the start of medication and disappeared after their withdrawal, and reappeared after repeated administration 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 histological examination skin, which will help to differentiate from diseases similar in appearance (eczema, psoriasis, cutaneous 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 symptoms of SLE may not be, but when they are examined, autoimmune antibodies are detected.

    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 systemic diseases connective tissue:
    • 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 there is a replacement normal tissue connective tissue (non-functional), 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- defeat various glands(tear, 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 С). Moderate prolonged fever (up to 39 0 С).
    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, omission is often detected upper eyelid(ptosis).
    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 work disruption intestinal tract(motor skills 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;
    • in defeat respiratory muscles- shortness of breath, up to complete paralysis of the muscles and respiratory arrest;
    • in defeat chewing 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 life threatening 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 quantity shaped elements blood;
    • 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 early age, though rarely the largest number cases of this disease occur 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 course diseases , 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 state of shock DIC - disseminated intravascular coagulation;
    • systemic lupus erythematosus in children often occurs in the form of vasculitis – inflammation 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. The skin may have various rashes 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 movements, starting with the small joints of the fingers.
    4. For children characterized by the formation of exudative pleurisy(liquid in pleural cavity), pericarditis (fluid in the pericardium, the membrane 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. 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 are more likely to 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. Nobody knows for what way will go autoimmune process.
      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 lethal outcome women.
      • And if the pregnancy did occur in period of 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 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 the death of a woman from kidney and / or heart failure, because it is on these bodies goes a huge burden 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: don’t overwork, don’t 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 postpartum period 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, 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 secreting contact way pathogen transmission.

      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 quantity 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. Products 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 medication, then the patient is recommended frequent fractional nutrition according to therapeutic diet- table number 1. All anti-inflammatory drugs are best taken with or immediately after meals.

      Treatment of systemic lupus erythematosus at home possible only after selection individual scheme therapy in a hospital and 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 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 kept 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. Except skin changes, this patient shows thickening of the joints of the phalanges of the fingers - signs of arthritis.

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

      Lupus lesions of the oral mucosa . By clinical picture 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.

    serious disease, during which the human immune system perceives the cells of its own body as foreign. This disease is terrible for its complications. Almost all organs suffer from the disease, but the musculoskeletal system and kidneys are the most affected (lupus arthritis and nephritis).

    Causes of systemic lupus erythematosus

    The history of the name of this disease goes back to a time when attacks by wolves on people were not rare, especially on cabbies and coachmen. At the same time, the predator tried to bite on the unprotected part of the body, most often on the face - nose, cheeks. As you know, one of bright symptoms disease is the so-called lupus butterfly- bright pink spots that affect the skin of the face.

    Experts came to the conclusion that women are more prone to this autoimmune disease: 85 - 90% of cases of the disease occur in the fair sex. Most often, lupus makes itself felt in the age range from 14 to 25 years.

    Why does systemic lupus erythematosus, is still completely unclear. But scientists still managed to find some regularities.

    • It has been established that people who, for various reasons, have to spend a lot of time in adverse temperature conditions (cold, heat) get sick more often.
    • Heredity is not the cause of the disease, but scientists suggest that relatives of the sick person are at risk.
    • Some research shows that systemic lupus erythematosus- This is the response of immunity to numerous irritations (infections, microorganisms, viruses). Thus, malfunctions in the work of immunity do not occur by chance, but with constant negative impact on the body. As a result, the body's own cells and tissues begin to suffer.
    • There is an assumption that certain chemical compounds can lead to the onset of the disease.

    There are factors that can provoke an exacerbation of an already existing disease:

    • Alcohol and smoking have a detrimental effect on the entire body as a whole and on the cardiovascular system in particular, and it already suffers from lupus.
    • Taking drugs containing large doses of sex hormones can cause an exacerbation of the disease in women.

    Systemic lupus erythematosus - the mechanism of the development of the disease

    The mechanism of the development of the disease is still not fully understood. It is hard to believe that the immune system, which is supposed to protect our body, is starting to attack it. According to scientists, the disease occurs when the regulatory function of the body fails, as a result of which certain types of lymphocytes become overly active and contribute to the formation of immune complexes(large protein molecules).

    Immune complexes begin to spread throughout the body, penetrating into various organs and small vessels, which is why the disease is called systemic.

    These molecules are attached to tissues, after which the release from them begins. aggressive enzymes. Being normal, these substances are enclosed in microcapsules and are not dangerous. But free, unencapsulated enzymes begin to destroy healthy body tissues. Numerous symptoms are associated with this process.

    The main symptoms of systemic lupus erythematosus

    Harmful immune complexes with blood flow spread throughout the body, so any organ can be affected. However, the person does not associate the first symptoms that appeared with such serious illness, How systemic lupus erythematosus because they are characteristic of many diseases. So, the following signs appear first:

    • unreasonable rise in temperature;
    • chills and muscle pain, fatigue;
    • weakness, frequent headaches.

    Later, there are other symptoms associated with the defeat of a particular organ or system.

    • One of the obvious symptoms of lupus is the so-called lupus butterfly - rash and flushing(overflow of blood vessels) in the cheekbones and nose. In fact, this symptom of the disease appears only in 45-50% of patients;
    • a rash can occur on other parts of the body: arms, abdomen;
    • another symptom may be partial hair loss;
    • ulcerative lesions of the mucous membranes;
    • the appearance of trophic ulcers.

    Lesions of the musculoskeletal system

    It suffers much more often than other tissues in this disorder. Most patients complain of the following symptoms.

    • Pain in the joints. Note that most often the disease affects the smallest. There are lesions of paired symmetrical joints.
    • Lupus arthritis, despite the similarity with it, differs from it in that it does not cause destruction bone tissue.
    • Approximately 1 in 5 patients develop deformity of the affected joint. This pathology is irreversible and can only be treated surgically.
    • In the stronger sex with systemic lupus, inflammation most often occurs in sacroiliac joint. Pain syndrome occurs in the coccyx and sacrum. Pain can be both permanent and temporary (after physical exertion).

    Damage to the cardiovascular system

    In about half of patients, a blood test reveals anemia, as well as leukopenia and thrombocytopenia. Sometimes it leads to drug treatment illness.

    • During the examination, the patient may show pericarditis, endocarditis, or myocarditis that has arisen for no apparent reason. No concomitant infections that could lead to damage to the heart tissue are detected.
    • If the disease is not diagnosed in time, then in most cases the mitral and tricuspid valves of the heart are affected.
    • Besides, systemic lupus erythematosus is a risk factor for the development of atherosclerosis, like other systemic diseases.
    • The appearance of lupus cells (LE-cells) in the blood. These are modified white blood cells that have been exposed to immunoglobulin. This phenomenon vividly illustrates the thesis that the cells of the immune system destroy other tissues of the body, mistaking them for foreign ones.

    Kidney damage

    • For acute and subacute lupus arises inflammatory disease of the kidneys, which is called lupus nephritis, or lupus nephritis. At the same time, fibrin deposition and the formation of hyaline thrombi begin in the kidney tissues. At untimely treatment going on a sharp decline kidney function.
    • Another manifestation of the disease is hematuria(the presence of blood in the urine), not accompanied by pain and not disturbing the patient.

    If the disease is detected and treated on time, then an acute kidney failure develops in about 5% of cases.

    Nervous System Damage

    • Delayed treatment can cause severe disorders of the nervous system in the form of convulsions, sensory disturbances, encephalopathy and cerebrovasculitis. Such changes are persistent and difficult to treat.
    • Symptoms manifested by the hematopoietic system. The appearance of lupus cells (LE-cells) in the blood. LE cells are leukocytes in which the nuclei of other cells are found. This phenomenon just vividly illustrates how the cells of the immune system destroy other tissues of the body, mistaking them for foreign ones.

    Diagnosis of systemic lupus erythematosus

    If a person is found at the same time 4 signs of illness he is diagnosed with: systemic lupus erythematosus. Here is a list of the main symptoms that are analyzed in the diagnosis.

    • The appearance of a lupus butterfly and a rash in the cheekbones;
    • increased skin sensitivity to sun exposure (redness, rash);
    • sores on the mucous membrane of the nose and mouth;
    • inflammation of two or more joints (arthritis) without bone damage;
    • inflamed serous membranes(pleurisy, pericarditis);
    • protein in the urine (more than 0.5 g);
    • dysfunction of the central nervous system (convulsions, psychosis, etc.);
    • found on blood test low maintenance leukocytes and platelets;
    • antibodies to their own DNA are detected.

    Treatment of systemic lupus erythematosus

    It should be understood that this disease is not treated for any specific period of time or with the help of surgery. This diagnosis is made for life, however systemic lupus erythematosus- not a verdict. Timely diagnosis and properly prescribed treatment will help to avoid exacerbations and allow you to lead a full life. At the same time, there is important condition- You can not be in the open sun.

    Various agents are used in the treatment of systemic lupus erythematosus.

    • Glucocorticoids. First, a large dose of the drug is prescribed to relieve the exacerbation, later the doctor reduces the dosage. This is done to reduce the side effect, which adversely affects a number of organs.
    • Cytostatics - quickly remove the symptoms of the disease (short courses);
    • Extracorporeal detoxification - fine purification of the blood from immune complexes by transfusion;
    • Non-steroidal anti-inflammatory drugs. These funds are not suitable for long-term use because they are harmful to of cardio-vascular system and reduce testosterone production.

    Significant assistance in the complex treatment of the disease will be provided by a drug that includes and natural ingredient- drone. Biocomplex helps to strengthen defensive forces organism and cope with this complex disease. It is especially effective in cases where the skin is affected.

    Natural remedies for complications of lupus

    It is necessary to treat concomitant diseases and complications - for example, lupus nephritis. It is necessary to constantly monitor the condition of the kidneys, since this disease ranks first in cases of mortality in systemic lupus erythematosus.

    Equally important is the timely treatment of lupus arthritis and heart disease. In that invaluable help provide drugs such as Dandelion P And Plus.

    Dandelion P- it is a natural chondroprotector that protects joints from destruction, restores cartilage tissue, and also helps to lower blood cholesterol levels. It also helps to remove toxins from the body.

    Dihydroquercetin Plus- improves blood microcirculation, removes harmful cholesterol, strengthens the walls of blood vessels, making them more elastic.

    is a serious autoimmune disease that is dangerous for its complications. Do not despair, because such a diagnosis is not a sentence. Timely diagnosis and proper treatment will help you avoid exacerbations. Be healthy!

    USEFUL TO KNOW:

    ABOUT DISEASES OF THE JOINTS

    No one thinks about how to avoid pain in the joints - the thunder did not strike, why put a lightning rod. Meanwhile, arthralgia - this is the name of this type of pain - affects half of people over forty years old and 90% of those who are over seventy. So preventing joint pain is something to think about, even if you…

    Laboratory research

    General blood analysis
    . An increase in ESR is often observed in SLE, but this sign does not correlate well with disease activity. An unexplained increase in ESR indicates the presence of an intercurrent infection.
    . Leukopenia (usually lymphopenia) is associated with disease activity.
    . Hypochromic anemia is associated with chronic inflammation, hidden gastric bleeding, taking certain drugs. Often, mild or moderate anemia is detected. Severe Coombs-positive autoimmune hemolytic anemia is observed in less than 10% of patients.

    Thrombocytopenia is commonly found in patients with APS. Very rarely develops autoimmune thrombocytopenia associated with the synthesis of AT to platelets.
    . An increase in CRP is uncharacteristic; noted in most cases in the presence of concomitant infection. Moderate increase in CRP concentration (<10 мг/мл) ассоциируется с атеросклеротическим поражением сосудов.

    General urine analysis
    Proteinuria, hematuria, leukocyturia are detected, the severity of which depends on the clinical and morphological variant of lupus nephritis.

    Biochemical research
    Changes in biochemical parameters are nonspecific and depend on the predominant lesion of internal organs in different periods of the disease. Immunological studies
    . Antinuclear factor (ANF) is a heterogeneous population of autoantibodies that react with various components of the cell nucleus. ANF ​​is detected in 95% of SLE patients (usually in high titer); its absence in the vast majority of cases is evidence against the diagnosis of SLE.

    Antinuclear AT. AT to double-stranded (native) DNA (anti-DNA) are relatively specific for SLE; detected in 50-90% of patients ♦ AT to histones, more characteristic of drug-induced lupus. AT to 5m antigen (anti-Sm) are highly specific for SLE, but they are detected only in 10-30% of patients; AT to small nuclear ribonucleoproteins are more often detected in patients with manifestations of mixed connective tissue disease ♦ AT to Ro/SS-A antigen (anti-Ro/SSA) are associated with lymphopenia, thrombocytopenia, photodermatitis, pulmonary fibrosis, Sjögren's syndrome. AT to La/SS-B antigen (anti-La/SSB) is often found together with anti-Ro.

    APL, false positive Wassermann reaction, lupus anticoagulant and AT against cardiolipin are laboratory markers of APS.

    Other laboratory abnormalities
    Many patients have so-called lupus cells - LE (ot lupus erythematosus) cells (leukocytes that phagocytized nuclear material), circulating immune complexes, RF, but the clinical significance of these laboratory disorders is small. In patients with lupus nephritis, a decrease in the total hemolytic activity of complement (CH50) and its individual components (C3 and C4) is observed, which correlates with the activity of nephritis (especially the C3 component).

    Diagnostics

    For the diagnosis of SLE, the presence of one symptom of the disease or one identified laboratory change is not enough - the diagnosis is established on the basis of the clinical manifestations of the disease, laboratory and instrumental research data, and the classification criteria for the disease of the American Association of Rheumatologists.

    American Rheumatological Association Criteria

    1. Rash on the cheekbones: fixed erythema on the cheekbones, tending to spread to the nasolabial area.
    2. Discoid rash: erythematous raised plaques with adherent skin scales and follicular plugs; old lesions may have atrophic scars.

    3. Photosensitivity: A skin rash resulting from an unusual reaction to sunlight.
    4. Ulcers in the oral cavity: ulceration of the oral cavity or nasopharynx; usually painless.

    5. Arthritis: Non-erosive arthritis affecting 2 or more peripheral joints, presenting with tenderness, swelling, and effusion.
    6. Serositis: pleurisy (pleuritic pain, or pleural friction rub, or presence of pleural effusion) or pericarditis (confirmed by echocardiography or by auscultation of pericardial rub).

    7. Kidney damage: persistent proteinuria> 0.5 g / day or cylindruria (erythrocyte, hemoglobin, granular or mixed).
    8. CNS damage: convulsions or psychosis (in the absence of drugs or metabolic disorders).

    9. Hematological disorders: hemolytic anemia with reticulocytosis, or leukopenia<4,0х109/л (зарегистрированная 2 и более раза), или тромбоцитопения <100х109/л (в отсутствие приёма ЛС).

    10. Immunological disorders ♦ anti-DNA or ♦ anti-Sm or ♦ aPL: - increased level of IgG or IgM (AT to cardiolipin); - positive test for lupus anticoagulant using standard methods; - False-positive Wassermann reaction for at least 6 months in the absence of syphilis confirmed by the treponema pallidum immobilization test and the treponemal AT fluorescence adsorption test.
    11. ANF: increased titers of ANF (in the absence of taking drugs that cause lupus-like syndrome). SLE is diagnosed when 4 or more of the 11 criteria listed above are found.

    Diagnostic criteria for APS

    I. Clinical criteria
    1. Thrombosis (one or more episodes of arterial, venous or small vessel thrombosis in any organ).
    2. 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 ).

    II. Laboratory Criteria
    1. 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 6 weeks.
    2. Plasma lupus anticoagulant in 2 or more studies at least 6 weeks apart, defined as follows
    . prolongation of plasma clotting time in phospholipid-dependent coagulation tests;
    . no correction for prolongation of screening test clotting times in mixing tests with donor plasma;
    . shortening or correction of the lengthening of the clotting time of screening tests with the addition of phospholiiides;
    . exclusion of other coagulopathy. A certain APS is diagnosed based on the presence of one clinical and one laboratory criterion.

    If SLE is suspected, the following tests should be performed
    . a general blood test with the determination of ESR and counting the content of leukocytes (with a leukocyte formula) and platelets. immunological blood test with the definition of ANF. general urine analysis. chest x-ray
    . ECG, echocardiography.

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