Abstract
Primary myocardial disease is common in patients with systemic sclerosis even in the absence of pulmonary or systemic hypertension and without significant renal or pulmonary disease. When clinically evident, primary scleroderma myocardial disease may be a poor prognostic factor. An increasing body of evidence suggests that myocardial involvement is due, at least in part, to early abnormal vasospasm, with or without associated structural abnormalities, of the small coronary arteries or arterioles.
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