Immunoglobulin G4-mediated sclerosing cholangitis as a risk factor for cholangiocarcinoma: A case report

Karin E Koopman, Elisabeth Bloemena, Geert Kazemier, Michael Klemt-Kropp

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Immunoglobulin (Ig)G4-mediated disease is a systemic autoimmune disease, which occasionally presents solely as sclerosing cholangitis (SC). IgG4-mediated SC is challenging to diagnose, as it may mimic cholangiocarcinoma radiologically, and carcinoma cells may produce IgG4. The diagnosis of IgG4-mediated disease is based on histological consensus criteria and response to corticosteroids. In addition to the radiological and histological overlap between IgG4-mediated SC and cholangiocarcinoma, IgG4-mediated SC may be considered as a risk factor for the development of cholangiocarcinoma. We herein present the case of a patient in whom cholangiocarcinoma developed in two lesions previously characterized as IgG4-mediated SC, including a suggested mechanism underlying the contribution of IgG4-mediated SC to the development of cholangiocarcinoma.

Original languageEnglish
Pages (from-to)786-788
Number of pages3
JournalJournal of Clinical Oncology
Issue number6
Publication statusPublished - Dec 2016

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