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Korean J Med > Volume 87(2); 2014 > Article
The Korean Journal of Medicine 2014;87(2): 182-186.
6년간의 자연 경과를 관찰할 수 있었던 IgG4 연관 경화성 담관염
김태오1, 김명환1, 최준혁1, 임두호1, 박상우1, 최준호1, 김진희2
1울산대학교 의과대학 서울아산병원 내과
2울산대학교 의과대학 서울아산병원 영상의학과
A Case of IgG4-Related Sclerosing Cholangitis with a 6-year Natural Clinical Course
Tae-Oh Kim1, Myung-Hwan Kim1, Joon Hyuk Choi1, Doo-Ho Lim1, Sangwoo Park1, Jun-Ho Choi1, Jin Hee Kim2
1Departments of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
2Departments of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
Corresponding Author: Myung-Hwan Kim ,Tel: +82-2-3010-3183, Fax: +82-2-476-0824, Email: mhkim@amc.seoul.kr
Received: December 26, 2013;   Revised: January 10, 2014;   Accepted: January 24, 2014.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a rare disease characterized by increased serum levels of IgG4 and dense infiltration of IgG4-positive plasma cells with fibrosis in the bile duct wall. IgG4-SC is frequently associated with autoimmune pancreatitis (AIP) and typically shows an excellent response to steroid therapy. Despite recent progress in understanding the clinical presentation of IgG4-SC, its diagnosis still remains a challenge, particularly if it manifests as an isolated hilar stricture with normal serum IgG4 concentrations. In this article, we report the case of a 75-year-old man with IgG4-SC in whom it was difficult to distinguish hilar cholangiocarcinoma due to the normal serum IgG4 concentration and no accompanying AIP. He had an indolent clinical course and showed slow progression of a bile duct lesion over 6 years.
Keywords: Immunoglobulin G4; Sclerosing cholangitis; Cholangiocarcinoma
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