Diffuse connective tissue diseases. Modern pictures are of clinic, diagnostics. Доц. Н.З.Ярема

Lupus erythematosus /LE/  LE is the system disease of connecting tissue, which develops on the basis of the genetically conditioned imperfection of immunoregulatory processes of, which results in formation of great number of antibodies to the own cages and their components and to the origin of immunocomplex inflammation with the damage of many organs and systems.

Diagnostic criteria of LE 1. Erythema is a “butterfly” 1. Erythema is a “butterfly” 2. Diskoid LE 2. Diskoid LE 3. Photosensibilisation 3. Photosensibilisation 4. Ulcers of cavity of mouth 4. Ulcers of cavity of mouth 5. Arthritis 5. Arthritis 6. Serositis 6. Serositis Pleuritis, pericarditis Pleuritis, pericarditis 7. Nephritis (proteinuriya, cylindruria) 7. Nephritis (proteinuriya, cylindruria) 8. Changes of the nervous system (neuropathy,psycosis) 8. Changes of the nervous system (neuropathy,psycosis) 9. Haematological changes(anaemia, leucopenia, trombocytopenia, lymphopenia) 9. Haematological changes(anaemia, leucopenia, trombocytopenia, lymphopenia) 10. Immunological changes(anti-DNC,anti- SM,antiphospholipid antibodies) 10. Immunological changes(anti-DNC,anti- SM,antiphospholipid antibodies) 11. Antinuclear antibodies 11. Antinuclear antibodies

СЧВ

Diagnosis The diagnosis of LE is set at presence of 4 and anymore from 11 criteria The diagnosis of LE is set at presence of 4 and anymore from 11 criteria ( recommendations of ACR, 1997 ) ( recommendations of ACR, 1997 )

skin “butterfly”

Systemic sclerosis / SSD/ Systemic sclerosis / SSD/  SSD is a system disease of connective tissue, for which making progress fibrosis and defeat of vessels is characteristic on the type of obliterate microangiopathy, which results in development of general syndrome of Reynoud, indurative changes of skin, defeats of the muscles and bones system, and internal organs( lungs, heart, kidneys, digestive tract)

Diagnostic criteria of SSD ACR, 1980 A.Main / large/ criteria A.Main / large/ criteria Proxymal sclerosis: symmetric bulge, drew on that induration of skin of fingers ( the defeats of extremities, face, neck, trunk are possible) Proxymal sclerosis: symmetric bulge, drew on that induration of skin of fingers ( the defeats of extremities, face, neck, trunk are possible)

Criteria of diagnostics of SSD B. Small criteria: B. Small criteria: Sclerodactylia Sclerodactylia Osteolisis of finger-tips is as a result of ischemia · Osteolisis of finger-tips is as a result of ischemia Fibrosis of basale parts of lungs · Fibrosis of basale parts of lungs At presence of one large or two small criteria set a diagnosis At presence of one large or two small criteria set a diagnosis

CREST - syndrome C- is a calcinosis of the skin C- is a calcinosis of the skin R- is a syndrome of Reynoud R- is a syndrome of Reynoud E- is a esophagitis E- is a esophagitis S- is a sclerodactylia S- is a sclerodactylia T- is a teleangioectasia T- is a teleangioectasia

Dermatomyositis/Polymyositis/ /DM/ Polymyositis is a system inflammatory disease with the overwhelming defeat of skeletal musculature, smooth musculature and skin Polymyositis is a system inflammatory disease with the overwhelming defeat of skeletal musculature, smooth musculature and skin Diagnostic criteria: Diagnostic criteria: 1. Weakness in the muscles of hands, feet, trunk 1. Weakness in the muscles of hands, feet, trunk 2. Increase of creatinphosphocinase or aldolase 2. Increase of creatinphosphocinase or aldolase 3. Spontaneous muscle pains 3. Spontaneous muscle pains 4. Changes are on an electromyogrames 4. Changes are on an electromyogrames 5. A positive test on antibodies to RNA 5. A positive test on antibodies to RNA 6. Undestructive arthritises and arthralgii 6. Undestructive arthritises and arthralgii 7. Signs of systemic inflammation 7. Signs of systemic inflammation 8. Results of the biopsy 8. Results of the biopsy Presence 4 of more than 4 criteria show diagnosis DM Presence 4 of more than 4 criteria show diagnosis DM

Diagnostic criteria of DM 1. Heliotrope pouring(purple red eritema of overhead eyelid)out 1. Heliotrope pouring(purple red eritema of overhead eyelid)out 2. Sign of Gottrona(atrophy eritema surface of joints of fingers) 2. Sign of Gottrona(atrophy eritema surface of joints of fingers) 3. Eritema of skin surface of joints of hands and feet 3. Eritema of skin surface of joints of hands and feet

Polyarteritis nodosa /PN/  PN- necrotic inflammation of middle and shallow arteries without glomerulonephritis or vasculitis arterioles, capillaries and venules.

Diagnostic criteria of PN 1. Loss of mass of body of 4kg and anymore 1. Loss of mass of body of 4kg and anymore 2. Livedo reticularis 2. Livedo reticularis 3. Pain or a sensitiveness is enhanceable in testicles 3. Pain or a sensitiveness is enhanceable in testicles 4. Diffuse myalgia, sensitiveness of muscles shins 4. Diffuse myalgia, sensitiveness of muscles shins 5. Mono- or polyneuropathy 5. Mono- or polyneuropathy 6. Diastolic pressure more than 90 mm Hg 6. Diastolic pressure more than 90 mm Hg

Diagnostic criteria of PN /continuation/ 7. Increase of level of urea or kreatinine 7. Increase of level of urea or kreatinine 8. Virus of hepatitis B 8. Virus of hepatitis B 9. Arteriographic change 9. Arteriographic change 10. Changes are at a biopsy 10. Changes are at a biopsy At presence of 3 or more criteria can be put diagnosis of PN At presence of 3 or more criteria can be put diagnosis of PN