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102P Systemic sclerosis, commonly called scleroderma, CREST syndrome, Raynaud phenomenon 

Kevin Mangum, D.O.
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Although commonly called scleroderma, this disorder is better labeled systemic sclerosis (SS), because it is characterized by excessive fibrosis throughout the body and not just the skin. Cutaneous involvement is the usual presenting symptom and eventually appears in approximately 95% of cases, but it is the visceral involvement-of the gastrointestinal tract, lungs, kidneys, heart, and skeletal muscles-that produces the major morbidity and mortality.
SS can be classified into two groups based on its clinical course:
Diffuse scleroderma, characterized by initial widespread skin involvement, with rapid progression and early visceral involvement.
Limited scleroderma, with relatively mild skin involvement, often confined to the fingers and face. Involvement of the viscera occurs late, and hence the disease in these patients generally has a fairly benign course. This is also called the CREST syndrome because of its frequent features of calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia.

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16 сен 2024

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