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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 • 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
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
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
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
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
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
Survival by decade Modified from Wallace
SLE treatment Modified from Wallace
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
Scleroderma survival-Effect of Lung Disease No lung dx UIP PAH
Diseased organ system or event leading to death in patients with systemic sclerosis (dSSc).
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
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