LARGE VESSEL OCCLUSIVE DISEASE-ASSOCIATED WITH CREST SYNDROME AND SCLERODERMA

Objectives-To report the cases of three patients with CREST syndrome a nd one patient with diffuse scleroderma who had severe macrovascular d isease and only minimal vascular risk factors. Methods-The medical his tories, physical examinations, and results of clinical investigations were reviewed in four patients. Results-These four patients had severe morbidity from macrovascular disease of the arms and legs in the pres ence of minimal underlying vascular risk factors. These patients repre sent 11% of the women with scleroderma seen at our hospital since 1974 . This is a greater than threefold increase above the expected proport ion of symptomatic vascular disease seen in population studies. In the patients with CREST syndrome, large vessel disease was first seen mor e than 10 years after the onset of Raynaud's phenomenon, which was the first manifestation of the disease. A pathological specimen of the ul nar artery from one patient showed severe luminal narrowing by an acel lular material with no evidence of atheroma. Conclusions-These cases s uggest an association of both the CREST syndrome and scleroderma with macrovascular disease.