and the Kidney硬皮病肾ppt课件.ppt

临床医学于波心房选择性钠通道阻滞剂在房颤中的应用颅内非肿瘤性病变的影像学表现基础医学慢性鼻窦炎鼻息肉围手术期综合治疗的临床指引泌尿生殖道感染衣原体检测和支原体培养及药敏结果分析 Scleroderma and the Kidney David Shure July 14, 2009 DD: Renal Failure ATN Renal Scleroderma Crisis Epidemiology Prevalence: 19-75 cases per 100,000 Susceptibility Age: peak occurrence 35-65 F:M 7-12:1 Environmental Factors: infection Occupational exposure: silica dust Usually occurs w/in 4-5 yrs of SSc onset Local vs Systemic Disease Classification dcSSC: diffuse cutaneous SSc lcSSc: limited cutaneous SSC SSc sine scleroderma – internal organ involvement Environmentally induced scleroderma Overlap syndrome: ie MCTD Calcinosis cutis Raynaud Phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia Pathogenesis Complex and poorly understood Immune activation Inflammatory response Vascular damage Excessive synthesis EC matrix and collagen deposition Hypothesis: interplay between early immunological events and vascular changes leading to generation of a pop of activated fibrogenic fibroblasts believed to be effector cells in disease Vascular and Endothelial Changes Appear to precede other features of SSc Vasoconstriction ET-1: significantly elevated in SSc and assoc with pulm htn, may initiate fibrosis Superoxide anions: released from endothelium neutralize NO Defective vasculogenesis: fewer circulating endothelial ell precursors ie CD34+, CD133+ and VEGF type 2 Pts with SSc have incr in endothelial cell surface expression of adhesion molecules and elevation in circulating levels of their soluble forms. Cytokine induced upregulation of adhesion molecules ie ICAM-1, VCAM-1, ELAM-1 Autoantibodies 75% pts with SSc have circulating autoantibodies Topoisomerase I (anti-scl 70) anti-topoisomerase antibodies highly specific for SSc, and correlate with greater risk for ILD and more extensive skin involvement Centromere antigens (12-44%) Anticentromere antibodies assoc with limited cutaneous involvement Fibrosis Fibrosis gradually replaces vascular inflammatory phase and ulti