The Korean journal of internal medicine

The Korean Association of Internal Medicine (대한내과학회)
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Immunoglobulin G4-related sclerosing cholangitis

  • Goodchild, George (Department of Gastroenterology, University College Hospital) ;
  • Pereira, Stephen P. (Department of Gastroenterology, University College Hospital) ;
  • Webster, George (Department of Gastroenterology, University College Hospital)
  • Received : 2018.01.09
  • Accepted : 2018.02.06
  • Published : 2018.09.01
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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.

Keywords

Acknowledgement

Supported by : National Institute for Health Research (NIHR)

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