Microvascular involvement in scleroderma is well recognized. Macrovascular disease is not. OBJECTIVE. To test the hypothesis that the prevalence of macrovascular disease is increased in patients with limited scleroderma (systemic sclerosis, lSSc). METHODS. A retrospective cohort study design was employed in which the prevalence of macrovascular disease in all female patients from specified hospitals (1974-90) with lSSc of at least 5 years' duration was compared and contrasted with that in a comparable group of controls. Each control was matched to one lSSc case by sex; age (+/- 5 yrs); number of inpatient admissions (+/- 2); year of last hospital admission (+/- 2 yrs), history of hypertension, cigarette smoking and diabetes mellitus, and medical record number most closely approximating the case. The distribution of vascular disease was assessed in the peripheral, coronary, and cerebral arterial territories. RESULTS. Peripheral macrovascular disease (PVD) occurred in 18 (58%) of the lSSC patients and only 3 (9.6%) of the controls (RR = 6.0; 95% CI 2.0-18). Of the 18 lSSc cases, 8 had PVD documented angiographically, 4 by arterial Doppler ultrasound, and 6 had absent peripheral pulses. Five of these required subsequent partial limb amputation. Two of the 3 controls with PVD had absent peripheral pulses, and 1 had an angiographically documented abdominal aortic aneurysm. No control required limb amputation. There was no significant difference in the prevalence of coronary artery or cerebrovascular disease between the groups. CONCLUSION. The prevalence of peripheral large vessel occlusive disease is increased in lSSC and associated with severe morbidity.