A Review of IgG4-related Sclerosing Cholangitis (IgG4-SC)

Neil C Khoury; John W Birk

doi:10.1097/MCG.0000000000001984

A Review of IgG4-related Sclerosing Cholangitis (IgG4-SC)

J Clin Gastroenterol. 2024 Feb 23. doi: 10.1097/MCG.0000000000001984. Online ahead of print.

Authors

Neil C Khoury¹, John W Birk

Affiliation

¹ Division of Gastroenterology and Hepatology, University of Connecticut Health, Farmington, CT.

PMID: 38385591
DOI: 10.1097/MCG.0000000000001984

Abstract

IgG4-related sclerosing cholangitis (IgG4-SC) is a unique, steroid-responsive, fibroinflammatory condition that is more commonly found in older men and is strongly associated with autoimmune pancreatitis (AIP). It may pose a diagnostic challenge at times due to closely mimicking hepatobiliary diseases such as primary sclerosing cholangitis (PSC), secondary sclerosing cholangitis (SSC), and cholangiocarcinoma. IgG4-SC has an excellent prognosis when promptly diagnosed and treated with steroids. Literature search strategy: The authors searched PubMed and Google Scholar, for articles with the search terms "autoimmune cholangiopathy" and "IgG4 cholangiopathy."