IgG4-Related Sclerosing Cholangitis Showing Natural Course of Progression to Decompensated Liver Cirrhosis / 대한췌담도학회지
Korean Journal of Pancreas and Biliary Tract
;
: 179-183, 2017.
Article
in Korean
| WPRIM
| ID: wpr-180596
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Plasma Cells
/
Prognosis
/
Fibrosis
/
Immunoglobulins
/
Cholangitis, Sclerosing
/
Cholangitis
/
Follow-Up Studies
/
Natural History
/
Inflammation
/
Liver
Type of study:
Observational study
/
Prognostic study
Limits:
Humans
Language:
Korean
Journal:
Korean Journal of Pancreas and Biliary Tract
Year:
2017
Type:
Article
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