59 - SCLERODERMA

Summary

Scleroderma (progressive systemic sclerosis) is a multisystem connective tissue disorder characterized by inflammation, fibrosis, and vasculopathy of affected tissues. CNS vasculitis, segmental vasospasm, and cerebrovascular calcifications may all play a role in causing strokes in patients with scleroderma. CNS vasculitis has been diagnosed in several patients with scleroderma and has been posited to cause strokes. Cerebral infarction in scleroderma patients in the absence of other plausible, causative factors should prompt an aggressive workup for vasculitis including angiography. Results of cerebral angiography in several patients thought to have vasculitis are consistent with the diagnosis of vasoconstriction. Arteriography revealed segmental, often smoothly contoured, narrowing of arteries of multiple sizes (small, medium, and large) in both the anterior and posterior circulations. Whether vascular calcium deposits were responsible for the patients' cerebrovascular symptoms is speculative. Scleroderma patients with cerebrovascular disease must take into consideration the potential causes of stroke.