Wegener씨 육아종증 1례

A Case of Wegener's Granulomatosis

Wegener써 육아종증은 1939년 Wegener가 \circled1 상하호흡기의 혈관염 및 괴사성 육아종, \circled2 전신적인 혈관염, \circled3 국한된 괴사성 사구체신염을 특징으로하는 질병을 Rhinogenic Granulomatosis라고 명명한데서 유래되었다. 초기에 이 질환은 몇달내에 사망하는 것으로 알고 있다. 그러나 최근 이 질환의 한정된 형(limited form)은 전형적인 형(classic form)에 비해 일반적으로 양성인 경과를 한다고 인지 되였다. 병리학적으로 이 질환은 한번의 조직생검으로 진단하기 힘들며 간혹 부검에서 확진되는 경우도 많다고 한다. 본교실에서는 최근 심한 시력장애, 청력장애를 일으킨 Wegener씨 육아종증 1례를 경험하였기에 이에 문헌고찰과 더불어 보고하는 바이다.

Wegener's granulomatosis is characterized by 3 criteria: 1. Necrotizing granulomas with vasculitis of upper and lower respiratory tracts 2. A systemic vasculitis 3. Focal necrotizing glomerulitis. This disease is one of the nonhealing disease in the otolaryngologic and ophthalmologic fields. A 48years old Korean male patient was seen with the complaints of nasal discharge, foul odor and frequent nasal bleeding. The patient was admitted after biopsy of the nasal cavity which diagnosed tuberculous granuloma, for biopsy of the maxillary sinus. After biopsy by Caldwell-Luc's approach this patient was complained with severe headache, visual impairment and cough. And so this patient was readmitted for further evaluation. Generally, the diagnosis was made after autopsy sometimes several years later after reevaluation of the case. Tuberculous granuloma was the pathological diagnosis on the basis of resected material in various cases. The correct diagnosis was made at autopsy occasionally. It is our intention to present this case with. literature review.