title: Immunoglobulin G4-related sclerosing cholangitis diagnosed by liver biopsy: a case report.
abstract: Patients with immunoglobulin (Ig) G4-related sclerosing cholangitis typically have a high serum IgG4 level. However, here we describe our experience of a patient with a normal serum IgG4 level for whom the cholangitis was diagnosed by liver biopsy. A 61-year-old male presented with elevated liver enzymes and a normal serum IgG4 level. The hilar, intrahepatic, and upper extrahepatic bile ducts were stenotic, with no evidence of a pancreatic lesion. We therefore performed a liver biopsy to differentiate between cholangiocarcinoma and primary sclerosing cholangitis. Pathological examination revealed lymphoplasmacytic infiltrates around the bile ducts with a storiform fibrosis. IgG4-positive plasma cells were also observed. These results fulfilled the Japanese diagnostic criteria for IgG4-related sclerosing cholangitis. When this condition is suspected, liver biopsy should be performed even when serum IgG4 levels are normal.
authors.affiliation: Department of Gastroenterology, Saiseikai Shiga Hospital.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Furuta
authors.forename: Hiroki
authors.initials: H
authors.lastname: Eguchi
authors.forename: Yoshiya
authors.initials: Y
authors.lastname: Takeda
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Fushiki
authors.forename: Takeshi
authors.initials: T
authors.lastname: Yasuda
authors.forename: Yuriko
authors.initials: Y
authors.lastname: Onozawa
authors.forename: Masanobu
authors.initials: M
authors.lastname: Katayama
authors.forename: Motoo
authors.initials: M
authors.lastname: Tanaka
authors.forename: Tadashi
authors.initials: T
authors.lastname: Shigematsu
authors.forename: Masamichi
authors.initials: M
authors.lastname: Bamba
mesh_terms: D001706:Biopsy; D049448:Cholangiopancreatography, Magnetic Resonance; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D002780:Cholestasis, Intrahepatic / Q000000981:diagnostic imaging / Q000209:etiology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed
chemical_list: D007074:Immunoglobulin G