Diffuzшс disease to connective tissue. The Differential diagnostics. Tactics squall

1. Diffuzшс disease to connective tissue. The Differential diagnostics. Tactics squall www.tma.uz

2. Diffuzic disease to connective tissue DBST- group nozology forms, characterizes system type of the inflammation different organ and systems, matching with development autumunal and immunizecomplex of the processes, and surplus fibroformation.

3. Diffuznye disease to connective tissue System red lupus System sclerodermic Idiopaticals inflammatory myopatic (dermatomyozit,polemyozit) Diffuznyy fasceit Polymyalgia rheumatical

4. Диффузные болезни соединительной ткани Disease SHegrena Relapsingpolyhondritis Relapsingpanniculitis Mixed disease to connective tissue Antifosfolipidnyy syndrome

6. Motivations for association in group DBST Similarity of the separate clinical manifestations in early stage disease and need of the undertaking dif.diagnostiki General laboratory factors to inflammatory activity

7. Motivations for association in group DBST General group and typical of each disease immunologic markers Close principles antiinflammatoryimmunosupersistic of the treatment Using extracorporemethods at crisis

8. Connective tissue Connective tissue (ST) presents itself united system regardless of localizations and specialization

9. Connective tissue consists From celluar element Intercellularmatrixcis

10. The most important hutches ST Fibroblasty Hondroblasty Osteoblasty Sinoviocity Obese hutches (labrosits)

11. Intercellular matrix includes Kollagen filament(supporting and fraudulent function) Retikulyr filament Elastic filament Main material, consisting of different proteoglycans

12. Functions ST 1.Suports function due to the fact that: ST forms • skeleton, • medicine to be taken externally covers (skin) • intercessor (framework) all organ and fabric

13. 2.Mechanicals function To account: Strong kologenycals of the filaments, Concentrations, Density of the packing Arhitektoniki, which provides resistance to voltage

14. 3.Trophic (metabolic) function To account: Celluar element Proteoglikanycal

15. 4.ЗProtections (barriers) function To account: Skin cover Seroznyh shell Capsules internal organ Makrofagycals Stranger of hutches (limfatikcels,plazmacels)

16. 5.Reparation function Consists in liquidations defect fabrics, appearing as a result Traumas Inflammations Breaches to circulations

17. Determination SRL SRL- system autoimmun rheumatic disease, developing on base genetic conditioned imperfection immunoregulytor processes, with formation ensemble antibody to own hutch origin immunokompleks inflammations, which effect is a damage many organ and systems (polyorgan insufficiency)

18. Etiology Etiology SRL remains unclear (genetic predisposition, infection, viruses, sexual hormones, factors surrounding ambiences) More than 70% fall ill at age 14-40 years, but peak to diseases accounts for 14-25 years Correlation of the mans and womans, illness SRL, forms from 1:8 before 1:10

19. The Categorization SRL (is designed by V.A. Nasonovoy 1972-1986y.) Depending on nature beginning: Under sharp current disease, aside from high temperature of the body, already at nearest months develops polysyndrom-picture with involvement in process vitally important organ and systems (sick remembers day begin disease)

20. The Categorization SRL (is designed by V.A. Nasonovoy 1986y.) Podostroe current is characterized undulation such symptoms, as fever, arthritis, polysyrozit, defeat of the skin. On length 1-1,5 years from first symptoms of the disease is formed polysystem, there is trend to progression, when during next intensification in pathological process are involved new organs and systems

21. The Categorization SRL (is designed by V.A. Nasonovoy 1986y.) Under chronic current on length of the many years disease runs monoolygosymptoms, amongst symptoms can be an arthritis, hematologyc of the breach, notsharp expressed proteinuria, defeat of the skin, epileptyforms fit

22. The Categorization SRL (is designed by V.A. Nasonovoy 1986y.) On degree of the activities disease in accordance with prononsed clinical symtoms and level of the laboratory factors Active: I - minimum, II - moderate, III - high Inactive: remission

23. Criteria American Revmatologic Assotiations (1982) Eritemes on cheek, on jugal salient Diskoidnye centres of the lupus Fotosensibilization Cankers in cavities oral or nose Nonerroziv arthritis Plevryt orperycardyt

24. Criteria American RevmatologicAssotiations (1982) 7. Persistycproteinuria more 0,5gr overnight or change urinary setting 8. Cricks or psychosises 9. Haemolytic anemia or leycopenia or trombocitopenia 10.Availability LE-hutches or antibody to DNK or falsepositive reaction Vasserman 11. Presence ANF Presence 4 signs does diagnosis reliable

25. Clinical manifestations SRL • Defeat of the skin beside 90% sick • Beside 20-25% sick skin syndrome (the erytematozeruption, discoid of the change) is an early sign disease. Beside 30% sick ulcer on mucous shell (painless) • Defeat supporting - a motor system Artralgii arthritises beside 90-100% sick Mialgii beside 35-45% sick • Defeat heart • Perikardit, Miokardit, LibmanEndokardit, Saks, Pankardit

26. Clinical manifestations SRL (continuation) Defeat light • 50-80% - dry and effusion pleurisy • Lupus pneumonit Defeat of the gastrointestinal tract in 50% events (vasculitmezenteryc container, autoimmun hepetitis) Defeat bud (50% events) • Nephritis with minimum urinary syndrome • Nephritis with nephrotyc syndrome • Beside 1/3 sick active lupus-nephritis with expressed by urinary syndrome

27. Clinical manifestations SRL (continuation) Defeat of the nervious system • Defeat CNS and periferyc of the nervious system 1/3 sick • Chorea, convulsive syndrome • Stubborn headaches, fits migraine, astenia, breaches VWVappear in the dreams • Psychosises (schizoprenic), psyhoemotion vagary • Affected syndrome, organic brain symptom

28. Clinical manifestations SRL (continuation) Hematologycchange Anemia chronicinflamation • Hemolitycanemia, retyculocytosi • Autoimun trombocitopenicpurpura, trombocitopenia • Leykopeniya with limphopenia

29. Diagnostics SRL For diagnosis SRL, aside from transfering clinical and laboratory sign, big importance has General weakness, malaise young age, feminine floor, stubborn fever fever, significant and quick loss of the mass of the body, escalated fallout hair

34. LABORATORY EXAMINATION • General blood test • General urinalysis • LE cells • Antinuklear antibodies • CIK • RF in small subtitle • EKG • EhoKG • Laboratory-instrumental examinations cardiovascular, respiratory, digestive, emiction and CNS • IgG, IgМ

35. TREATMENT GKS (prednizolon 50-60 mg\sut) before 6 -12 week Citostatic immunodepressor (azatioprin, ciklofosfan, metotreksat 2-2,5 mg\kg in day) 4-aminohinoline (delagil 0,25-0,5 g\d or plakvenil 0,2-0,4 g\d) Plazmoforezis Limfociteforezis Hemosorbtion Long receiving the aspirin (100 mg\d)

36. System sklerodermiasklerodermia. Determination System sklerodermia-autoimmun disease to connective tissue, main clinical signs are conditioned-wide-spreadbreach microcirculation -fibrozis of the skin and internal organ

37. System sklerodermia. Determination HVD-progressing disease with typical change the skin, supporting-motor device, internal organ with wide-spread vazospastyc breaches on type of the syndrome Rhine, in base which lies defeat to connective tissue with prevalence fibroz and container on type obliterationendoarteriolit.

38. Etiologiya HVD Etiologiya and patogenesysHVD it is not enough studied Women(woman)s are ill at the average 7 once more often, than men The Disease is more often revealled at age 30-50 years.

39. Categorization HVD 3 Clinical forms HVD select Depending on spread skin syndrome: Diffuznuyu sclerodermia-characterizationgeneraly by defeat of the skin and typical visceral by defeats (digestive tract, heart, light and buds)

40. Categorization HVD Limited sclerodermia (damage of the skin on cyst mainly and person) or CREST-syndrome, named in accordance with initial letter of main his(its) manifestations: kalcinozys, syndrome Rhine, ezofagyt, sclerodactelia, teleangiektazia

41. Categorization HVD Overlap or cross syndrome combination SSD with sign revmatyk, dermatomiosite of the arthritis or system red lupus

42. Diagnostic signs system sklerodermia srlerodermik defeat of the skin (skleroderma) -a syndrome Rhine, дигитальные язвочки/ribs оsteo-muscular syndrome (with contractura) osteolyze kalcinozy Basis pneumopfybrosys The Main signs:

45. При заполнении барием толстого кишечника у пациента с системной склеродермией выявляются дивертикулы с "широким ртом" (стрелки)