Korean J Intern Med.  2018 Sep;33(5):841-850. 10.3904/kjim.2018.018.

Immunoglobulin G4-related sclerosing cholangitis

Affiliations
  • 1Department of Gastroenterology, University College Hospital, London, UK. George.webster1@nhs.net

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.

Keyword

Immunoglobulin G4 related disease; Immunoglobulin G4 cholangiopathy; Autoimmune pancreatitis; Cholangitis, sclerosing; Biliary stricture
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