IgG4-Related Sclerosing Cholangitis Showing Natural Course of Progression to Decompensated Liver Cirrhosis.
10.15279/kpba.2017.22.4.179
- Author:
Jisoo HAN
1
;
Jae Hyuck JUN
;
Kyung Hwa JUNG
;
Dae Hyun JEONG
;
Jae Cheol PARK
;
Soomin NOH
;
Li Chang HSING
;
Myung Hwan 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;
Hepatic cirrhosis;
Cholangitis
- MeSH:
Cholangitis;
Cholangitis, Sclerosing*;
Fibrosis;
Follow-Up Studies;
Humans;
Immunoglobulins;
Inflammation;
Liver Cirrhosis*;
Liver*;
Natural History;
Plasma Cells;
Prognosis
- From:Korean Journal of Pancreas and Biliary Tract
2017;22(4):179-183
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Immunoglobulin G4-related disease (IgG4-RD) is characterized by extensive IgG4-positive plasma cells infiltration resulting in inflammation and fibrosis of the affected organs. Patient with IgG4-RD shows an excellent response to glucocorticoid therapy especially when given at early onset stages. IgG4-related sclerosing cholangitis (IgG4-SC) is considered biliary manifestation of IgG4-RD. The natural history and long-term prognosis of IgG4-SC is not well defined and most of the previous case series have reported short duration of follow-up. We present the case of development of decompensated liver cirrhosis from IgG4-related sclerosing cholangitis with a 6-year natural clinical course.
