The CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) is a variant but not a benign form of scleroderma. Pulmonary hypertension without pulmonary fibrosis appears to be more prevalent in the CREST than in scleroderma patients. Moreover, pulmonary hypertension in CREST may be rapidly progressive and a cause of sudden death from severe cor pulmonale.
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