Open AccessImmunoglobulin G4-mediated sclerosing cholangitis as a risk factor for cholangiocarcinoma: A case report
Author(s) -
Karin E. Koopman,
Elisabeth Bloemena,
Geert Kazemier,
Michael Klemt-Kropp
Publication year - 2016
Publication title -
molecular and clinical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.442
H-Index - 7
eISSN - 2049-9469
pISSN - 2049-9450
DOI - 10.3892/mco.2016.1040
Subject(s) - medicine , igg4 related disease , antibody , disease , cancer , oncogene , pathology , primary sclerosing cholangitis , immunology , cell cycle
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.