A controlled clinicopathologic study of myocardial fibrosis in systemic sclerosis (scleroderma)

Clinicopathologic correlations of myocardial fibrosis were examined in 54 autopsied patients with scleroderma and 54 age and sex matched autopsy controls. Thirty eight (70%) of the patients with scleroderma had myocardial fibrosis compared to 20 (37%) of the controls (p less than 0.005). There was no significant difference in the prevalence of contraction band necrosis in the patients with scleroderma (22%) compared to controls (17%). Patients with scleroderma with left ventricular dysfunction in the absence of other causative factors clinically had a greater prevalence of both advanced myocardial fibrosis (60%) and contraction band necrosis (40%) than did the other patients with scleroderma or the controls. We conclude that patients with scleroderma with the greatest likelihood of advanced myocardial fibrosis can be identified clinically, and their findings are consistent with the presence of microvascular coronary vasospasm, a "myocardial Raynaud's phenomenon."