폐출혈 및 뇌의 출혈성 종괴를 동반한 Wegener씨 육아종증 1 예

Abstract

Wegener`s granulomatosis is a necrotizing, granulomatous vasculitis that involves multiple organs including the upper and lower respiratory tract and the kidney. The anitineutrophilic cytoplasmic antibody is a serologic marker with high sensitivity and specificity, which is useful in diagnosis of the disease and is considered to have a significant role in its pathogenesis. Wegener`s granulomatosis had been a fatal disease until combination therapy with cyclophosphamide and corticosteroids was introduced, which have improved the clinical course and the survival significantly. Wide spectrum of neurologic involvements such as peripheral and cranial neuritis, myelopathy, and cerebritis in Wegener`s granulomatosis has been reported, but manifestation of mass-like formation in the brain has rarely been reported. We experienced a case of Wegener`s granulomatosis with pulmonaty hemorrhage, renal failure, and a hemorrhagic mass in the brain. Its diagnosis was based on clinical findings, positive result of ANCA test, and the thoracoscopic lung biopsy. The hemorrhagic mass of the brain was found by magnetic resonance imaging and it is considered to be the result of granulomstous inflammation and vasculitis. With the combination therapy the cerebral lesion was slightly improved but the patient expired due to irreversible renal failure and progressive pulmonary hemorrhage.