LIMITED SCLERODERMA IS ASSOCIATED WITH INCREASED PREVALENCE OF MACROVASCULAR DISEASE

Microvascular involvement in scleroderma is well recognized. Macrovasc ular disease is not, Objective, To test the hypothesis that the preval ence of macrovascular disease is increased in patients with limited sc leroderma (systemic sclerosis, ISSc).Methods, A retrospective cohort s tudy design was employed in which the prevalence of macrovascular dise ase in all female patients from specified hospitals (1974-90) with lSS c of at least 5 years' duration was compared and contrasted with that in a comparable group of controls. Each control was matched to one ISS c case by sex; age (+/-5 yrs); number of inpatient admissions (+/-2); year of last hospital admission (+/-2 yrs), history of hypertension, c igarette 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 territ ories. 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% CI2.0-18). Of the 18 ISSc cases, 8 had PVD documented angiograph ically, 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 req uired limb amputation. There was no significant difference in the prev alence of coronary artery or cerebrovascular disease between the group s. Conclusion, The prevalence of peripheral large vessel occlusive dis ease is increased in ISSC and associated with severe morbidity.