Hypereosinophilia with IgG4 Associated Cholangiopathy

Abstract

IgG4-related sclerosing cholangitis (IgG4-SC) belongs to the spectrum of IgG4-related disease which encompasses many medical conditions. We report a case of a 59-year-old man who presented with clinical and radiologic features of primary sclerosing cholangitis but where hypereosinophilia along with an extremely high blood level of IgG4, liver infiltration with IgG4-positive plasma cell and a spectacular response to corticoids treatment, confirmed the diagnosis of IgG4-SC.