title: Primary sclerosing cholangitis with elevated serum IgG4 levels and/or infiltration of abundant IgG4-positive plasma cells.
abstract: Immunoglobin G4-related sclerosing cholangitis (IgG4-SC) is recognized as one of the systemic sclerosing diseases characterized by abundant IgG4-positive plasma cells with effective steroid therapy. On the other hand, primary sclerosing cholangitis (PSC), recognized as a sclerosing cholangitis of unknown origin without steroid efficacy, has been often clinically confused with IgG4-SC. To date, the prognosis of IgG4-SC is unclear, while the prognosis of PSC is well known to be poor. Therefore, it is clinically very important to be able to distinguish IgG4-SC from PSC. However, at the present time it still remains unclear whether PSC may sometimes be misdiagnosed as IgG4-SC or not. Herein, we report three rare cases of PSC with elevated serum IgG4 levels and/or an infiltration of abundant IgG4-positive plasma cells in the liver: a young male with ulcerative colitis (UC), and elderly female and a young female, each with elevated serum IgG4 levels. The first two patients showed infiltration of abundant IgG4-positive plasma cells in the portal area of the liver without response to steroid therapy. From our experiences, we emphasize that some patients with PSC, who do not respond to steroid therapy, show elevated serum IgG4 levels and/or infiltration of abundant IgG4-positive plasma cells, although the mechanism still remains unclear.
authors.affiliation: The Third Department of Internal Medicine, Division of Gastroenterology and Hepatology, Kansai Medical University, Moriguchi, Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Koyabu
authors.forename: Kazushige
authors.initials: K
authors.lastname: Uchida
authors.forename: Norimasa
authors.initials: N
authors.lastname: Fukata
authors.forename: Takeo
authors.initials: T
authors.lastname: Kusuda
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Ikeura
authors.forename: Yutaku
authors.initials: Y
authors.lastname: Sakaguchi
authors.forename: Katsunori
authors.initials: K
authors.lastname: Yoshida
authors.forename: Masaaki
authors.initials: M
authors.lastname: Shimatani
authors.forename: Toshiro
authors.initials: T
authors.lastname: Fukui
authors.forename: Mitsunobu
authors.initials: M
authors.lastname: Matsushita
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Uemura
authors.forename: Masaki
authors.initials: M
authors.lastname: Kaibori
authors.forename: Makoto
authors.initials: M
authors.lastname: Takaoka
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Nishio
authors.forename: Kazuichi
authors.initials: K
authors.lastname: Okazaki
mesh_terms: D000328:Adult; D000368:Aged; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D003093:Colitis, Ulcerative / Q000150:complications; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D008099:Liver / Q000276:immunology* / Q000378:metabolism; D008297:Male; D010950:Plasma Cells / Q000276:immunology / Q000378:metabolism; D011379:Prognosis; D017211:Treatment Failure; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G