Korean J Pancreas Biliary Tract.  2017 Oct;22(4):179-183. 10.15279/kpba.2017.22.4.179.

IgG4-Related Sclerosing Cholangitis Showing Natural Course of Progression to Decompensated Liver Cirrhosis

• Han J
• Jun JH
• Jung KH
• Jeong DH
• Park JC
• Noh S
• Hsing LC
• Kim MH

• KMID: 2448615
• DOI: http://doi.org/10.15279/kpba.2017.22.4.179

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.

Keyword

Immunoglobulin G4; Hepatic cirrhosis; Cholangitis

MeSH Terms

Cholangitis
Cholangitis, Sclerosing*
Fibrosis
Follow-Up Studies
Humans
Immunoglobulins
Inflammation
Liver Cirrhosis*
Liver*
Natural History
Plasma Cells
Prognosis
Immunoglobulins