성문하 협착을 동반한 베게너 육아종증 1예

Abstract

Wegener`s granulomatosis (WG) is a multisystemic inflammatory disease characterized by necrotizing granulomatous inflammation and vasculitis of unknown etiology which classically affects the upper airway, lung, and kidney. Subglottic stenosis (SGS) is rare and occurs independently of other features of active WG. SGS can be a life-threatening manifestation of disease, and prompt diagnosis is essential. Since the presenting symptoms of SGS can be nonspecific, SGS should be considered in the differential diagnosis of any patient with WG who has increasing dyspnea, voice change, or cough. Although both surgical and medical treatments have been utilized, the optimal therapeutic approach to SGS in patients with WG has not been determined. So far, a case of WG with SGS has not been reported in Korea. Recently, we experienced a case of 59-year-old woman with SGS in WG. She responded to prednisolone and methotrexate therapy and did not require surgical intervention. So we report this case with a review of literatures.