Department faculty and hospital therapy of medical faculty and department internal diseases of medical prophylactic faculty. Systemic lupus erythematosis. Department faculty and hospital therapy of medical faculty and department internal diseases of medical prophylactic faculty. Systemic lupus erythematosis.

Systemic connective tissue disorders 1. Systemic lupus erythematosis 2. Systemic scleroderma 3. Dermatomyositis 4. Diffuse fasciitis 5. rheumatic Polymyalgia 6. Recurrent cellulitis 7. Relapsing polychondritis 8. Sjogren's syndrome and disease 9. Mixed connective tissue disease 10. Antiphospholipid syndrome

Systemic lupus erythematosus (SLE) Chronic disease is mostly polysyndromic young women and girls, against a background of growing genetically imperfect due to immune regulatory processes, leading to uncontrolled production of antibodies to its own cells and their components, with the development of autoimmune and immune complex chronic inflammation. Chronic disease is mostly polysyndromic young women and girls, against a background of growing genetically imperfect due to immune regulatory processes, leading to uncontrolled production of antibodies to its own cells and their components, with the development of autoimmune and immune complex chronic inflammation.

EPIDEMIOLOGY Annually fall ill in different regions of 1chelovek Annually fall ill in different regions of 1chelovek The disease most often develops in women (10-20:1) The disease most often develops in women (10-20:1) Identify new cases per year per 1 million population Identify new cases per year per 1 million population 70% of the sick at the age of years, the "peak" at years 70% of the sick at the age of years, the "peak" at years

Etiology Unsettled suggested that some microorganisms, toxic solid matter and drugs may be responsible for the development of SLE, but direct evidence of involvement of - or a particular factor has not yet been received. On the role of genetic factors indicate greater concordance for SLE in monozygotic than in bizygotic twins and the prevalence of SLE (5-12%) among blood relatives of patients. Unsettled suggested that some microorganisms, toxic solid matter and drugs may be responsible for the development of SLE, but direct evidence of involvement of - or a particular factor has not yet been received. On the role of genetic factors indicate greater concordance for SLE in monozygotic than in bizygotic twins and the prevalence of SLE (5-12%) among blood relatives of patients.

Pathogenesis Due to several closely related-governmental mechanisms: a polyclonal (B cell), the late Ab- specific (T cell) activation of the immune system, defects in apoptosis lymphocytes etc. Effector mechanisms that determine the damage to internal organs associated with the first turn humoral, cellular and not with immune reactions. As the SLE develops mainly in women of reproductive age, there is no doubt an important role in the regulation of hormonal pathogenesis of the disease. Due to several closely related-governmental mechanisms: a polyclonal (B cell), the late Ab- specific (T cell) activation of the immune system, defects in apoptosis lymphocytes etc. Effector mechanisms that determine the damage to internal organs associated with the first turn humoral, cellular and not with immune reactions. As the SLE develops mainly in women of reproductive age, there is no doubt an important role in the regulation of hormonal pathogenesis of the disease.

Patomorphology In areas of damage to the connective tissue determine the amorphous mass of solid matter of nuclear, stained with hematoxylin in blue purple (hematoxylin bodies). Neutrophils swallowed such cells in vitro is called LE cells. Immune complexes in connective tissue and vascular walls consisting of DNA-DNA antibodies and components of a compliment, painted (and fibrin), eosin, forming a picture of fibrynoid necrosis.

Clinical manifestations of SLE Skin lesions - 90% of patients In 20-25% of patients with cutaneous syndrome is an early sign of disease. The defeat of support - the motor system Arthralgia - arthritis in % of patients Myalgia - at 35-45% of patients Defeat heart Pericarditis Myocarditis Endocarditis Libman - Sacks Endopericarditis Pericarditis Myocarditis Endocarditis Libman - Sacks Endopericarditis

Clinical manifestations of SLE (continued) Defeat light 50-80% - dry and swampy pleurisy Lupus pneumonitis The defeat of the gastrointestinal tract - in 50% Kidney damage Nephritis with minimal urinary syndrome Nephritis with nephrotic syndrome In one third of patients with active lupus nephritis, with marked urinary syndrome

Clinical manifestations of SLE (continued) Defeat nervous system Involvement of the central and peripheral nervous system - 1 / 3 of patients Involvement of the central and peripheral nervous system - 1 / 3 of patients Chorea Chorea Convulsions Convulsions Mental changes Mental changes Affective syndrome Affective syndrome Organic brain symptoms Organic brain symptoms

Laboratory examination General analysis blood General analysis blood Urinalysis Urinalysis LE cells LE cells Antinuclear antibodies Antinuclear antibodies CEC CEC RF small credits RF small credits ECG ECG EchoCS EchoCS Laboratory and instrumental examinations of cardiovascular, respiratory, digestive, urinary and central nervous system Laboratory and instrumental examinations of cardiovascular, respiratory, digestive, urinary and central nervous system IgG, IgM IgG, IgM

Diagnostic criteria 1. Rashes on the cheeks (lyupoid butterfly ") fixed eruption (flat or raised), which has a tendency to spread to the nasolabial area 2. Discoid rash erythematous raised patches with scales and surrounding follicular plugs, the old centers may be atrophic scars 3. Photosensitivity skin rash resulting from skin exposure of sunlight (in history or physician observation)

Diagnostic criteria (continued) 4. Erosion and ulcers in the mouth ulcerations of the mouth or throat, usually painless (to register a physician) 4. Erosion and ulcers in the mouth ulcerations of the mouth or throat, usually painless (to register a physician) 5. Arthritis nonerosive arthritis of 2 or more peripheral joints, manifested by soreness, swelling and effusion 5. Arthritis nonerosive arthritis of 2 or more peripheral joints, manifested by soreness, swelling and effusion 6. Serozity pleuritis: pleural pain, pleural friction rub and / or the presence of pleural effusion, pericarditis: documented by echocardiography, or listen to your doctor pericardial friction sound 6. Serozity pleuritis: pleural pain, pleural friction rub and / or the presence of pleural effusion, pericarditis: documented by echocardiography, or listen to your doctor pericardial friction sound

Diagnostic criteria (continued) 7. Renal disease-resistant proteinuria greater than 0.5 g / day or cylinders (erythrocytic, tubular, granular, mixed), hematuria 7. Renal disease-resistant proteinuria greater than 0.5 g / day or cylinders (erythrocytic, tubular, granular, mixed), hematuria 8. CNS convulsions - in the absence of medication or metabolic disorders (uremia, ketoacidosis, electrolyte imbalance), psychosis - in the absence of medication or electrolyte imbalance 8. CNS convulsions - in the absence of medication or metabolic disorders (uremia, ketoacidosis, electrolyte imbalance), psychosis - in the absence of medication or electrolyte imbalance 9. Hematological disorders leucopenia below 4 × 10 9 / L, recorded at least 2 times, lymphohenia less than 1.5 x 10 9 / L, recorded at least 2 times, thrombocytopenia less than 100 × 10 9 / L unrelated to medication 9. Hematological disorders leucopenia below 4 × 10 9 / L, recorded at least 2 times, lymphohenia less than 1.5 x 10 9 / L, recorded at least 2 times, thrombocytopenia less than 100 × 10 9 / L unrelated to medication

Diagnostic criteria (continued) 10. Immunological detection of disorders of lupus coagulant, false-positive Wasserman background for at least 6 months in places without sobject with syphilis reactions and pale treponema immobilization test fluorescent treponemal antibody absorbtion 10. Immunological detection of disorders of lupus coagulant, false-positive Wasserman background for at least 6 months in places without sobject with syphilis reactions and pale treponema immobilization test fluorescent treponemal antibody absorbtion 11. Antinuclear antibody titer increased their revealed by immunofluorescence or similar method without taking drugs and causing lupus like syndrome 11. Antinuclear antibody titer increased their revealed by immunofluorescence or similar method without taking drugs and causing lupus like syndrome

Clinical and laboratory characteristics of the active disease process in SLE Index The degree of activity IIIIII Body temperature 38 ° C and above Less than 38 ° C Normal Loss of fleshExpressedModerateSlight Нарушение трофики ExpressedModerate /Slight The skin lesion"Butterfly" and lupus erythema-type capillarytis Nonspecific erythema Discoid pockets PolyarthritisAcute, subacute SubacuteDeforming, arthralgia

Clinical and laboratory characteristics of the active disease process in SLE (continued) PericarditisSwampyDryAdhesive MyocarditisPronouncedModerateCardiosclerosis Endocarditis Libman - Sacks Pronounced _failure mitral valve PleurisySwampyDryAdhesive Diffuse glomerulonephritis Nephrotic syndromeMixed type Uric syndrome Defeat nervous system Ensefalo radiculoneuritis EnsefaloneuritisPolyneuritis

Clinical and laboratory characteristics of the active disease process in SLE (continued) Hemoglobin g / LLess than or more ESR mm / hr45 or more Fibrinogen g / l6 or more54 γ-globulin,% LE cells51-2Single or missing ANF ​​ titers 128 and above6432 Type of emissionMarginalHomogeneousor marginal Homogeneous Antibodies to DNAPronouncedModerateSlight

Working classification of clinical variants of the flow in SLE (Nasonova V.A ) Flow pattern Phase and level of activity Clinical and morphological characteristics of lesions SkinJointsSerous membra nes HeartLungsKidneyNervous system Acute Sub- acute Chro- nic Phase: Active The degree of activity: high (III) Moderate (II) Min (I) Phase: Inactive Symptom Butter- flies Erythema, cutaneous purpura Discoid lupus. Arthral -gia Poly- arthritis Polysero citis: pleurisy, peri- carditis Myocar ditis Endoca rditis Pneumo nitis, Pneumo fibrosis Lupus nephritis (with nephrotic syndrome, isolated urinary syndrome ) Meningo- lopoli- radiculon euritis, polyneuro pathy

Differential diagnosis Unsettled for at least 40 diseases similar to SLE, especially at the onset of the disease. 1. rheumatoid arthritis 2. Acute rheumatic fever 3. systemic scleroderma 4. systemic vasculitis

Treatment glucocorticosteroids intensive care pulse therapy combined pulse therapy glucocorticosteroids intensive care pulse therapy combined pulse therapy 4 - Amino quinolinedrugs Delagil Plaquenil 4 - Amino quinolinedrugs Delagil Plaquenil NSAIDs NSAIDs Extracorporeal therapies plasmapheresis immunosorbtion Extracorporeal therapies plasmapheresis immunosorbtion Software management of patients with SLE Software management of patients with SLE

FORECAST Currently, the prognosis of SLE patients has improved substantially. Survival of patients with SLE 10 years after diagnosis 80% after 20 years - 60%. Nevertheless, mortality of SLE patients is 3 times higher than in the population. Currently, the prognosis of SLE patients has improved substantially. Survival of patients with SLE 10 years after diagnosis 80% after 20 years - 60%. Nevertheless, mortality of SLE patients is 3 times higher than in the population.