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Duke PA Scleroderma

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Scleroderma is a Rheumatic disease characterized by __   fibrosis (thickening) of the skin (scleroderma) and internal organs (lungs, kidneys, heart, GI tract)  
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vascular dysfunction associated with scleroderma   Raynaud’s phenomenon, digital ischemia, infarction  
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scleroderma is __ times more common in woment   3-4  
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onset of scleroderma is unlikely before decades __   3-4  
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environmental factors implicated in scleroderma   viral infection, silica  
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earliest manifestation of scleroderma   Raynaud's phenomenon  
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results in endothelial injury→ increased permeability, abnormal smooth muscle cell proliferation and platelet aggregationbasement membrane thickens and fibrosis of the adventitial layers developsaffects capillaries, arterioles and even large vessels   Raynaud's phenomenon  
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the tissue fibrosis in scleroderma is brought on by   vasculopathy and autoimmunity  
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characterized by progressive replacement of normal tissue with dense connective tissue→ exuberant matrix remodeling and scar formation   scleroderma fibrosis  
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__skin, lungs, tendon sheaths, heart, GI tract and some endocrine organsleads to functional impairment   tissue fibrosis develops in  
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sclerodactyly   thickened fingers  
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