Clinical clues to suspicion of IgG4‐associated sclerosing cholangitis disguised as primary sclerosing cholangitis or hilar cholangiocarcinoma

Abstract

ISC should be considered in the differential diagnosis of hilar/intrahepatic biliary strictures. Past or concurrent AIP or extrabiliary organ involvement strongly suggests the possibility of ISC. Significant infiltration of IgG4-positive cells on endobiliary or liver biopsy specimens, and/or elevated serum IgG4 levels, highly support the diagnosis of ISC and provide the rationale for steroid therapy.