Cerebral vasculitis in an adolescent with juvenile rheumatoid arthritis

A 16-year-old girl with longstanding polyarticular juvenile rheumatoid arthritis was referred from Chuuk State, Federated States of Micronesia, for evaluation and management of weakness and joint pains. She had a right hemiparesis with central facial weakness, polyarticular arthritis, and a dense cataract of the left eye with phthisis bulbi. Extensive evaluation using magnetic resonance angiography revealed vasculitis of the anterior and middle cerebral arteries. She was treated with "pulse" doses of methylprednisolone intravenously and she improved remarkably. Repeat angiography demonstrated dramatic improvement of the vasculitis. There is a paucity of literature regarding cerebral vasculitis in juvenile rheumatoid arthritis. That literature is reviewed, and our patient is discussed with regard to the few published reports.