Journal of Clinical Otolaryngology Head and Neck Surgery
The Busan, Ulsan, Gyeoungnam Branch of Korean Society of Otolaryngology-Head and Neck Surgery
증례

안면 마비와 난청으로 진단된 면역글로불린 G4 연관질환 동반 육아종성 다발 혈관염 1예

김형식1, 두전강1, 여승근1, 김상훈1,*
Hyung-sik Kim1, Jeon Gang Doo1, Seung Geun Yeo1, Sang Hoon Kim1,*
1경희대학교 의과대학·의학전문대학원 이비인후과학교실
1Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyung Hee University, Seoul, Korea
*교신저자: 김상훈, 02447 서울 동대문구 경희대로 26 경희대학교 의과대학·의학전문대학원 이비인후과학교실 전화 :(02) 958-8474·전송 :(02) 958-8470 E-mail: hoon0700@naver.com

© Copyright 2018 The Busan, Ulsan, Gyeoungnam Branch of Korean Society of Otolaryngology-Head and Neck Surgery. This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Received: Aug 03, 2018; Revised: Sep 27, 2018; Accepted: Nov 28, 2018

Published Online: May 31, 2020

ABSTRACT

Granulomatosis with polyangiitis (GPA) and Immunoglobulin (Ig) G4-related disease (IgG4-RD) are rare diseases and early diagnosis and proper management are imperative to prevent multi-organ damage. The authors present a case of a 60 years old woman who had facial paralysis and hearing loss. Lt intact canal wall tympanomastoidectomy, Lt facial nerve decompression and ossiculoplasty with partial ossicular replacement prosthesis (PORP) was done. During operation, middle ear tissue was biopsied and GPA with IgG4-RD was diagnosed. After methyl prednisolone (MPD) pulse therapy and azathioprine therapy, the severity of paralysis was improved. We present this case because common otologic symptoms like facial palsy and hearing loss could be initial symptoms of rare systemic disease. (J Clinical Otolaryngol 2018;29:240-244)

Keywords: 육아종증; 면역글로불린 G; 얼굴마비
Keywords: Granulomatosis with polyangiitis; Immunoglobulin G; Facial nerve palsy