Central Nervous System Vasculitis in Rheumatoid Arthritis: A Case Report - Abstract
Central nervous system vasculitis is a rare and extremely severe manifestation of rheumatoid arthritis. We report a case of cerebral vasculitis in a 42-year-old woman with a longstanding deforming and erosive rheumatoid arthritis and extra articular manifestations. She had a previous history of granulomatous pleuritis treated with tuberculostatics without response, and multiple pulmonary complications interpreted as pneumonias and asthma exacerbations. This leaded to under treatment, avoiding methotrexate or other highly effective anti-rheumatic drugs, and using only hydroxychloroquine, prednisone and non-steroidal anti-inflammatory drugs most of the time. She was admitted for respiratory symptoms. Chest computed tomography revealed reticulonodular pulmonary involvement. She was treated with hydrocortisone and broad spectrum antibiotics, and showed a favorable response, but afterwards she suffered a seizure. Magnetic resonance imaging of the brain with T2 and FLAIR sequences showed multiples bilateral hyperintense signal images, suggestive of hemispheric vasculitis. Magnetic resonance angiography was normal. She received phenytoin and immunosuppressive treatment with methylprednisolone pulses, tapering dose of oral prednisone and a 6-month course of intravenous cyclophosphamide, followed by oral azathioprine for 2 years along with low dose aspirin. Treatment was then switched to methotrexate and afterwards leflunomide was added. Brain and thoracic images improved remarkably, and she remained neurologically asymptomatic and without respiratory symptom at a 4-year follow-up. This case illustrates how aggressive rheumatoid arthritis can be if and the importance of differential diagnosis between infectious and inflammatory manifestations, as treatment may be completely different. It also shows that cerebral rheumatic vasculitis may have a good response to immunosuppressive therapy.