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http://dx.doi.org/10.3904/kjim.2018.018

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)
Publication Information
The Korean journal of internal medicine / v.33, no.5, 2018 , pp. 841-850 More about this Journal
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
Immunoglobulin G4 related disease; Immunoglobulin G4 cholangiopathy; Autoimmune pancreatitis; Cholangitis, sclerosing; Biliary stricture;
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