A Case of Wegener's Granulomatosis with Periaortitis and Pachymeningitis

Abstract

Wegener’s grandulomatosis (WG) is a systemic small-vessel vasculitis characterized by necrotizing granulomatous lesions and anti-neutrophil cytoplasmic antibodies (ANCA). WG frequently affects the upper and lower respiratory tracts and kidney but involvement of the pachymeninges or large vessels has rarely been reported. We report the first case of WG with periaortitis and pachymeningitis in Korea. A 57-year-old female complained of a stabbing headache, paresthesia below the thoracic 4th nerve level, and lower extremity weakness. During her course, she developed otitis media with sensorineural hearing loss, nodular scleritis, and microhematuria. A neuroimaging study showed an intradural mass in the area from the cervical 7th spine to the thoracic 5th spine, left cerebral convexity, and the left petrous area. A chest computed tomography scan revealed an enhanced and thickened aortic arch. A dural biopsy showed necrotizing granulomatous inflammation, and anti-MPO ANCA was positive. Under a diagnosis of WG, she received high-dose glucocorticoid and oral cyclophosphamide with improvement.