A Case of IgG4-Related Sclerosing Cholangitis with a 6-year Natural Clinical Course.
10.3904/kjm.2014.87.2.182
- Author:
Tae Oh KIM
1
;
Myung Hwan KIM
;
Joon Hyuk CHOI
;
Doo Ho LIM
;
Sangwoo PARK
;
Jun Ho CHOI
;
Jin Hee KIM
Author Information
1. Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea. mhkim@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Immunoglobulin G4;
Sclerosing cholangitis;
Cholangiocarcinoma
- MeSH:
Aged;
Bile Ducts;
Cholangiocarcinoma;
Cholangitis, Sclerosing*;
Constriction, Pathologic;
Diagnosis;
Fibrosis;
Humans;
Immunoglobulin G;
Immunoglobulins;
Pancreatitis;
Plasma Cells;
Rare Diseases
- From:Korean Journal of Medicine
2014;87(2):182-186
- CountryRepublic of Korea
- Language:Korean
-
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.
