Immunoglobulin G4-related sclerosing cholangitis.
- Author:
George GOODCHILD
1
;
Stephen P PEREIRA
;
George WEBSTER
Author Information
- Publication Type:Review
- Keywords: Immunoglobulin G4 related disease; Immunoglobulin G4 cholangiopathy; Autoimmune pancreatitis; Cholangitis, sclerosing; Biliary stricture
- MeSH: Cholangiocarcinoma; Cholangitis, Sclerosing*; Diagnosis, Differential; Immunoglobulins*
- From:The Korean Journal of Internal Medicine 2018;33(5):841-850
- CountryRepublic of Korea
- Language:English
- Abstract: Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition of which IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation. In this review, we provide an overview of IgG4-RD, with a focus on the biliary manifestations. In particular, we describe the important differential diagnoses of IgG4-SC, namely, primary sclerosing cholangitis and cholangiocarcinoma, outline diagnostic criteria for IgG4-SC, provide insight into possible pathophysiological mechanisms underlying the disease and discuss short and long-term management options of this recently described disease.
