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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