Arterial thrombosis in scleroderma

Abstract

Clinical and pathological observations are presented on five patients with scleroderma who developed thrombosis of a major vessel. Three died following intestinal infarction or limb gangrene and two had digit or extremity amputation. Severe intimal thickening of major arteries with thrombus formation was noted but no significant abnormality was found in the clotting activity of the blood. Histochemical studies in three cases suggested a reduction of fibrinolytic activity at the site of thrombosis in the diseased vessels. These studies support the view that the vascular system is profoundly altered in scleroderma and may play an important role in some manifestations of the disease.