Scleroderma and central nervous system vasculitis

We describe a patient with scleroderma (CREST syndrome) and central nervous system vasculitis. While angiography demonstrated segmental symmetrical arterial narrowing characteristic of vasculitis, results of leptomeningeal biopsy were normal. There was no evidence of systemic vasculitis, renal failure, or malignant hypertension previously thought to be required to explain central nervous system dysfunction in patients with scleroderma. Signs and symptoms attributable to vasculitis were reversible with aggressive immunosuppressive therapy.