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Immunoglobulin G4-Related Sclerosing Cholangitis

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Diseases of the Liver and Biliary Tree

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Abstract

IgG4-related sclerosing cholangitis (IgG4-SC) is a biliary tract manifestation of IgG4-related diseases (IgG4-RDs). A recent epidemiological study in Japan demonstrated that the point prevalence of IgG4-SC was 2.18 (95% confidence interval, 2.13–2.23) per 100,000 population. Although the etiology remains unclear, recent studies have attempted to clarify the roles of the IgG4 molecule and novel autoantibodies. IgG4-SC is clinically characterized by (1) chronic elevation of cholestatic liver enzyme levels in the sera, (2) significant elevation of serum IgG4 levels, (3) diffuse or segmental narrowing of the intra and/or extrahepatic bile ducts with thickening of the bile duct wall in imaging studies, (4) marked lymphoplasmacytic and IgG4-positive plasma cell infiltration and fibrosis in histological studies, (5) presence of IgG4-RD in other organs, mainly involving autoimmune pancreatitis, and (6) excellent response to corticosteroids. As no single diagnostic biomarker with high sensitivity and specificity is present, diagnostic criteria with a combination of these clinical parameters have been developed for the diagnosis of IgG4-SC. Although very challenging in most cases, differential diagnosis of IgG4-SC from primary sclerosing cholangitis or pancreatobiliary malignancies is extremely important because patients with IgG4-SC respond to corticosteroids very well, and long-term outcomes are expected to be excellent.

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  1. Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan

    Atsushi Tanaka

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Correspondence to Atsushi Tanaka .

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  1. Scientific Consultant, Scientific Institute for Research, Hospitalization and Healthcare (IRCCS) Negrar, Verona, Italy

    Annarosa Floreani

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Tanaka, A. (2021). Immunoglobulin G4-Related Sclerosing Cholangitis. In: Floreani, A. (eds) Diseases of the Liver and Biliary Tree. Springer, Cham. https://doi.org/10.1007/978-3-030-65908-0_10

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