Department faculty and hospital therapy of Department faculty and hospital therapy of medical faculty and department medical faculty and department

internal diseases of medical internal diseases of medical prophylactic faculty. prophylactic faculty.

Systemic lupus Systemic lupus erythematosis.erythematosis.

Systemic connective tissue disordersSystemic connective tissue disorders

1.1. Systemic lupus erythematosisSystemic lupus erythematosis2.2. Systemic sclerodermaSystemic scleroderma3.3. DermatomyositisDermatomyositis4.4. Diffuse fasciitisDiffuse fasciitis5.5. rheumatic Polymyalgiarheumatic Polymyalgia6.6. Recurrent cellulitisRecurrent cellulitis7.7. Relapsing polychondritisRelapsing polychondritis8.8. Sjogren's syndrome and diseaseSjogren's syndrome and disease9.9. Mixed connective tissue diseaseMixed connective tissue disease10.10. Antiphospholipid syndromeAntiphospholipid syndrome

Systemic lupus erythematosus Systemic lupus erythematosus (SLE)(SLE)

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

EPIDEMIOLOGYEPIDEMIOLOGY Annually fall ill in different regions of Annually fall ill in different regions of

1chelovek 400-20001chelovek 400-2000 The disease most often develops in women The disease most often develops in women

(10-20:1)(10-20:1) Identify 50-70 new cases per year per 1 Identify 50-70 new cases per year per 1

million populationmillion population 70% of the sick at the age of 14 - 40 years, the 70% of the sick at the age of 14 - 40 years, the

"peak" at 14 - 25 years"peak" at 14 - 25 years

EtiologyEtiology Unsettled suggested that some Unsettled suggested that some

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

PathogenesisPathogenesis Due to several closely related-governmental Due to several closely related-governmental

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

PatomorphologyPatomorphologyIn areas of damage to the connective tissue In areas of damage to the connective tissue

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

Clinical manifestations of SLEClinical manifestations of SLE

Skin lesions Skin lesions - 90% of patients- 90% of patientsIn 20-25% of patients with cutaneous syndrome is an In 20-25% of patients with cutaneous syndrome is an early sign of disease.early sign of disease.

The defeat of support The defeat of support - the motor system- the motor systemArthralgia - arthritis in 90-100% of patientsArthralgia - arthritis in 90-100% of patientsMyalgia - at 35-45% of patientsMyalgia - at 35-45% of patients

Defeat  heartDefeat  heart PericarditisPericarditis

MyocarditisMyocarditisEndocarditis Libman - SacksEndocarditis Libman - SacksEndopericarditisEndopericarditis

Clinical manifestations of SLE Clinical manifestations of SLE (continued)(continued)

Defeat lightDefeat light50-80% - dry and swampy pleurisy50-80% - dry and swampy pleurisy

Lupus pneumonitisLupus pneumonitisThe defeat of the gastrointestinal tract - in 50%The defeat of the gastrointestinal tract - in 50%

Kidney damageKidney damageNephritis with minimal urinary syndromeNephritis with minimal urinary syndromeNephritis with nephrotic syndromeNephritis with nephrotic syndromeIn one third of patients with active lupus nephritis, In one third of patients with active lupus nephritis, with marked urinary syndromewith marked urinary syndrome

Clinical manifestations of SLE Clinical manifestations of SLE (continued)(continued)

Defeat  nervous systemDefeat  nervous system Involvement of the central and peripheral Involvement of the central and peripheral

nervous system - 1 / 3 of patientsnervous system - 1 / 3 of patients ChoreaChorea ConvulsionsConvulsions Mental changesMental changes Affective syndromeAffective syndrome Organic brain symptomsOrganic brain symptoms

Laboratory examinationLaboratory examination General  analysis bloodGeneral  analysis blood UrinalysisUrinalysis LE cellsLE cells Antinuclear antibodiesAntinuclear antibodies CECCEC RF small creditsRF small credits ECGECG EchoCSEchoCS Laboratory and instrumental examinations of Laboratory and instrumental examinations of

cardiovascular, respiratory, digestive, urinary and cardiovascular, respiratory, digestive, urinary and central nervous systemcentral nervous system

IgG, IgMIgG, IgM

Diagnostic criteriaDiagnostic criteria1. Rashes on the cheeks (lyupoid butterfly ") 1. Rashes on the cheeks (lyupoid butterfly ")

fixed eruption (flat or raised), which has a fixed eruption (flat or raised), which has a tendency to spread to the nasolabial areatendency to spread to the nasolabial area

2. Discoid rash erythematous raised patches with 2. Discoid rash erythematous raised patches with scales and surrounding follicular plugs, the scales and surrounding follicular plugs, the old centers may be atrophic scarsold centers may be atrophic scars

3. Photosensitivity skin rash resulting from skin 3. Photosensitivity skin rash resulting from skin exposure of sunlight (in history or physician exposure of sunlight (in history or physician observation)observation)

Diagnostic criteria (continued)Diagnostic criteria (continued)

4. Erosion and ulcers in the mouth ulcerations of the 4. Erosion and ulcers in the mouth ulcerations of the mouth or throat, usually painless (to register a physician)mouth or throat, usually painless (to register a physician)

5. Arthritis nonerosive arthritis of 2 or more peripheral 5. Arthritis nonerosive arthritis of 2 or more peripheral joints, manifested by soreness, swelling and effusionjoints, manifested by soreness, swelling and effusion

6. Serozity pleuritis: pleural pain, pleural friction rub and 6. Serozity pleuritis: pleural pain, pleural friction rub and / or the presence of pleural effusion, pericarditis: / or the presence of pleural effusion, pericarditis: documented by echocardiography, or listen to your documented by echocardiography, or listen to your doctor pericardial friction sounddoctor pericardial friction sound

Diagnostic criteria (continued)Diagnostic criteria (continued)

7. Renal disease-resistant proteinuria greater than 0.5 g / 7. Renal disease-resistant proteinuria greater than 0.5 g / day or cylinders (erythrocytic, tubular, granular, mixed), day or cylinders (erythrocytic, tubular, granular, mixed), hematuriahematuria

8. CNS convulsions - in the absence of medication or 8. CNS convulsions - in the absence of medication or metabolic disorders (uremia, ketoacidosis, electrolyte metabolic disorders (uremia, ketoacidosis, electrolyte imbalance), psychosis - in the absence of medication or imbalance), psychosis - in the absence of medication or electrolyte imbalanceelectrolyte imbalance

9. Hematological disorders leucopenia below 4 × 10 9 / L, 9. Hematological disorders leucopenia below 4 × 10 9 / L, recorded at least 2 times, lymphohenia less than 1.5 x 10 recorded at least 2 times, lymphohenia less than 1.5 x 10 9 / L, recorded at least 2 times, thrombocytopenia less 9 / L, recorded at least 2 times, thrombocytopenia less than 100 × 10 9 / L unrelated to medicationthan 100 × 10 9 / L unrelated to medication

Diagnostic criteria (continued)Diagnostic criteria (continued)

10. Immunological detection of disorders of lupus 10. Immunological detection of disorders of lupus coagulant, false-positive Wasserman background for coagulant, false-positive Wasserman background for at least 6 months in places without sobject with at least 6 months in places without sobject with syphilis reactions and pale treponema immobilization syphilis reactions and pale treponema immobilization test fluorescent treponemal antibody absorbtiontest fluorescent treponemal antibody absorbtion

11. Antinuclear antibody titer increased their revealed 11. Antinuclear antibody titer increased their revealed by immunofluorescence or similar method without by immunofluorescence or similar method without taking drugs and causing lupus like syndrometaking drugs and causing lupus like syndrome

Clinical and laboratory characteristics of the Clinical and laboratory characteristics of the active disease process in SLEactive disease process in SLE

Index The degree of activity

III II I

Body temperature

38 ° C and above

Less than 38 ° C

Normal

Loss of flesh Expressed Moderate Slight

Нарушение трофики

Expressed Moderate Moderate/Slight

The skin lesion "Butterfly" and lupus erythema-type capillarytis

Nonspecific erythema

Discoid pockets

Polyarthritis Acute,  subacute

Subacute Deforming,arthralgia

Clinical and laboratory characteristics of the active disease Clinical and laboratory characteristics of the active disease process in SLE (continued)process in SLE (continued)

Pericarditis Swampy Dry Adhesive

Myocarditis Pronounced Moderate Cardiosclerosis

EndocarditisLibman - Sacks

Pronounced _ failuremitralvalve

Pleurisy Swampy Dry Adhesive

Diffuse glomerulonephritis

Nephrotic syndrome Mixedtype

Uricsyndrome

Defeatnervous system

Ensefalo radiculoneuritis

Ensefaloneuritis Polyneuritis

Clinical and laboratory characteristics of the active disease Clinical and laboratory characteristics of the active disease process in SLE (continued)process in SLE (continued)

Hemoglobin g / L Less than 100 100-110 120 or more

ESR mm / hr 45 or more 30-40 16-20

Fibrinogen g / l 6 or more 5 4

γ-globulin,% 30-35 24-25 20-23

LE cells 5 1-2 Single or missing

ANF titers 128 and above 64 32

Type of emission Marginal Homogeneousor marginal

Homogeneous

Antibodies to DNA Pronounced Moderate Slight

Working classification of clinical variants of the flow Working classification of clinical variants of the flow in SLE (Nasonova V.A. 1972 - 1986)in SLE (Nasonova V.A. 1972 - 1986)

Flow pattern

Phase and level of activity

Clinical and morphological characteristics of lesions

Skin Joints Serous membranes

Heart Lungs Kidney Nervous system

Acute Sub-acuteChro-nic

Phase:ActiveThe degree of activity:high (III) Moderate (II)Min (I)Phase: Inactive

Symptom Butter-fliesErythema,cutaneouspurpuraDiscoid lupus.

Arthral-gia

Poly-arthritis

Polyserocitis: pleurisy, peri-carditis

Myocarditis Endocarditis

Pneumonitis, Pneumofibrosis

Lupus nephritis (with nephrotic syndrome, isolated urinary syndrome)

Meningo-lopoli-radiculoneuritis, polyneuropathy

Differential diagnosisDifferential diagnosis

Unsettled for at least 40 diseases Unsettled for at least 40 diseases similar to SLE, especially at the onset similar to SLE, especially at the onset of the disease.of the disease.1. rheumatoid arthritis1. rheumatoid arthritis2. Acute rheumatic fever2. Acute rheumatic fever3. systemic scleroderma3. systemic scleroderma4. systemic vasculitis4. systemic vasculitis

TreatmentTreatment glucocorticosteroidsglucocorticosteroids

intensive careintensive carepulse therapypulse therapycombined pulse therapycombined pulse therapy

4 - Amino quinolinedrugs4 - Amino quinolinedrugs       Delagil       Delagil       Plaquenil       Plaquenil

NSAIDsNSAIDs Extracorporeal therapiesExtracorporeal therapies

plasmapheresisplasmapheresisimmunosorbtionimmunosorbtion

Software management of patients with SLESoftware management of patients with SLE

FORECASTFORECAST Currently, the prognosis of SLE Currently, the prognosis of SLE

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