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DU PA Scleroderma
Duke PA Scleroderma
| Question | Answer |
|---|---|
| Scleroderma is a Rheumatic disease characterized by __ | fibrosis (thickening) of the skin (scleroderma) and internal organs (lungs, kidneys, heart, GI tract) |
| vascular dysfunction associated with scleroderma | Raynaud’s phenomenon, digital ischemia, infarction |
| scleroderma is __ times more common in woment | 3-4 |
| onset of scleroderma is unlikely before decades __ | 3-4 |
| environmental factors implicated in scleroderma | viral infection, silica |
| earliest manifestation of scleroderma | Raynaud's phenomenon |
| 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 |
| the tissue fibrosis in scleroderma is brought on by | vasculopathy and autoimmunity |
| characterized by progressive replacement of normal tissue with dense connective tissue→ exuberant matrix remodeling and scar formation | scleroderma fibrosis |
| __skin, lungs, tendon sheaths, heart, GI tract and some endocrine organsleads to functional impairment | tissue fibrosis develops in |
| sclerodactyly | thickened fingers |