scleroderma and the kidney--Shure-7-14-09

scleroderma and the kidney--Shure-7-14-09 - Scleroderma and...

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Scleroderma and the Kidney David Shure July 14, 2009
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DD: Renal Failure ATN Renal Scleroderma Crisis
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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
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Local vs Systemic Disease
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Classification dcSSC: diffuse cutaneous SSc lcSSc: limited cutaneous SSC SSc sine scleroderma – internal organ involvement Environmentally induced scleroderma Overlap syndrome: ie MCTD
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Calcinosis cutis Raynaud Phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia
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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
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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
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scleroderma and the kidney--Shure-7-14-09 - Scleroderma and...

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