A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity

Ko, Seok Hwa; Yoon, Hyung Joon; Zheng, Tao; Jeong, Jin Hyeok

doi:2017.24.2.138

J Rhinol > Volume 24(2); 2017

Case Report

Journal of Rhinology 2017;24(2):138-142.
Published online November 30, 2017.
DOI: https://doi.org/10.18787/jr.2017.24.2.138

A Case of Immunoglobulin G4-Related Sclerosing Disease not Responded to Steroid in Nasal Cavity

Seok Hwa Ko, Hyung Joon Yoon, Tao Zheng, Jin Hyeok Jeong

Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Hanyang University, Seoul, Korea. ent@hanyang.ac.kr

스테로이드에 반응하지 않은 비강에 발생한 면역글로불린 G4 연관 경화성 질환 1례

고석화, 윤형준, 정타오, 정진혁

한양대학교 의과대학 이비인후-두경부외과학교실

Abstract

IgG4-related sclerosing disease was first discovered when researching autoimmune pancreatitis. It is an autoimmune disease that causes fibrosis on the pancreas, bile duct, gall bladder, salivary glands, and lungs. It is rarely reported in the nasal cavity. The diagnosis of IgG4-related sclerosing disease is based on biopsy findings demonstrating the characteristic histopathologic findings and immunohistochemical staining. IgG4-related sclerosing disease shows good response to high-dose steroid therapy. However, some patients do not respond to this treatment. Azathioprine or rituximab can be helpful in such case. We report our experience with IgG4-related sclerosing disease treated with azathioprine for 2 years, along with a related literature review.

Key Words: IgG4-related sclerosing disease;Plasma cell;Nasal cavity;Paranasal sinus