LARGE CEREBRAL VESSEL OCCLUSIVE DISEASE IN SYSTEMIC LUPUS-ERYTHEMATOSUS

We systematically investigated clinical, laboratory, radiologic, and p athologic features, including treatment and prognosis, of stroke syndr omes in 30 patients, six from our institution and 24 from the literatu re, with systemic lupus erythematosus (SLE) and symptomatic large cere bral vessel occlusive disease, documented by angiography or autopsy. T he average age at stroke onset was 35 years, and the diagnosis of SLE was made on average 4.4 years prior to that. At least 86% had active S LE at the time of their stroke. Headache was common at onset. We found major intracranial or extracranial vessel occlusive process by (1) th rombus, (2) dissection, (3) fibromuscular dysplasia or vasculitis, and (4) atherosclerosis. The presumed mechanisms were coagulopathy, cardi ogenic embolism, large cerebral vessel vasculitis or occlusive vasculo pathy, cervical arterial dissection, and premature atherosclerosis. Th e short-term death rate was 40% and the recurrent stroke rate was 13%. We conclude that symptomatic large cerebral vessel occlusive disease in SLE generally occurs several years after the diagnosis of SLE, usua lly during the active phase of the disease, is related to heterogeneou s mechanisms, and carries a relatively poor short-term outcome.