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CTD objectives

CTD objectives. Know why CTDs are considered autoimmune diseases Know what the epidemiology of CTDs implies about their pathophysiology Understand the concept of disease criteria Know the criteria for SLE and SSc Consider the role of immune pathways in autoimmune disease pathogenesis

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CTD objectives

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  1. CTD objectives • Know why CTDs are considered autoimmune diseases • Know what the epidemiology of CTDs implies about their pathophysiology • Understand the concept of disease criteria • Know the criteria for SLE and SSc • Consider the role of immune pathways in autoimmune disease pathogenesis • Review principals of therapy for SLE and PSS

  2. Connective Tissue Disease • Definitions • Connective tissue diseases are those syndromes manifest as multiple organ system involvement in which the pathogenesis is heavily dependent on self-directed immune mechanisms • Examples • SLE, MCTD, SS, PSS, polymyositis

  3. Connective tissue diseases (CTD) are characterized by specific properties • General systemic features-malaise, fever, weight loss • Evidence for multiple organ system involvement • Musculoskeletal involvement-arthritis, myalgia • Immune alterations-lead to pathologic inflammation • Therapeutic response to immunosuppression

  4. Malar rash Discoid rash Photosensitivity Oral ulcers Nonerosive arthritis Serositis Renal disorder Neurologic disorder Hematologic disorder Immunologic disorder Antinuclear antibody(ANA) 1997 update of the 82 revised criteria for SLE Must have 4 criteria Modified from the Primer

  5. SLE CRITERIA DEFINITIONS

  6. SLE criteria definitions

  7. SLE criteria definitions

  8. SLE criteria definitions

  9. Antinuclear antibodies (ANA) • 99% of SLE patients • 5% of normals • Prevalence is approximately =1/1000 women • Hence- if 1000 women are tested; 51 are ANA positive of which only 1 has SLE

  10. ANA profiles in connective tissue disease

  11. Malar rash of SLE

  12. Discoid rash

  13. SLE-nonerosive arthritis

  14. SLE onset by sex and age

  15. SLE by race

  16. Definite Ultraviolet light Drugs Sulfa antibiotics Probable Chemicals Hydralazine Procainamide Anti-TNF Hair dyes Lipstick Infections EBV Possible Smoke Vinyl chloride Asbestos Silicone Foods Hydrazines L-Canavanine LE disease triggers

  17. Survival by decade Modified from Wallace

  18. Serious or life-threatening consequences of SLE

  19. SLE treatment Modified from Wallace

  20. Cytotoxic medications

  21. Preliminary criteria for Scleroderma (PSS or SSc) • Major criteria • Proximal scleroderma • Minor criteria • Sclerodactyly • Digital pitting • Bibasilar pulmonary fibrosis 1 Major or two or more minor must be met. Modified from the Primer

  22. Clinical subsets of scleroderma

  23. Scleroderma antibodies

  24. SclerodermadSSc lSSc

  25. Sclerodactyly of hands

  26. Calcinosis cutis

  27. Scleroderma survival-Effect of Lung Disease No lung dx UIP PAH

  28. Diseased organ system or event leading to death in patients with systemic sclerosis (dSSc).

  29. dSSc GI Renal

  30. Treatment of sclerodermarenal crisis

  31. Treatment of scleroderma • Focus on symptoms • Antibiotics for bowel motility • Gloves for Raynaud's • Treat responsive syndromes • PAH with bosentan, flolan, Rovatio • Renal crisis with ACE inhibitors

  32. Summary • CTD are multi-system diseases • Immune mechanisms are key to their pathogenesis and therapy • Diagnosis are clinically based using criteria • Treatment is non-specific-aimed instead at specific manifestations • A move toward prevention of key complications like CAD and renal crisis is in progress

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