title: IgG4-related intrahepatic sclerosing cholangitis resulting in sepsis caused by secondary suppurative inflammation: report of a case.
abstract: We report a case of pre-disseminated intravascular coagulation caused by secondary suppurative inflammation in a patient with immunoglobulin (Ig) G4-related sclerosing cholangitis. The patient was a 78-year-old man in whom a localized stenosis of the intrahepatic bile duct was found without any other bile duct stricture or symptoms. He underwent surgical resection 6 months later for acute severe cholangitis and sepsis caused by bile duct obstruction. The resected specimen contained an abscess and nodulary mass in the liver. Immunohistochemical analysis revealed IgG4-positive plasma cell infiltration, whereby we diagnosed IgG4-related sclerosing cholangitis. As IgG4-related sclerosing cholangitis limited to within the intrahepatic portion is extremely rare, we present this case with a review of the literature.
authors.affiliation: Department of Organ Regulatory Surgery, Fukushima Medical University School of Medicine, 1 Hikarigaoka, Fukushima, 960-1295, Japan, bobibobu@yahoo.co.jp.
authors.forename: Motonobu
authors.initials: M
authors.lastname: Saito
authors.forename: Tatsuo
authors.initials: T
authors.lastname: Shimura
authors.forename: Yuko
authors.initials: Y
authors.lastname: Hashimoto
authors.forename: Teruhide
authors.initials: T
authors.lastname: Ishigame
authors.forename: Rei
authors.initials: R
authors.lastname: Yashima
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Koyama
authors.forename: Seiichi
authors.initials: S
authors.lastname: Takenoshita
mesh_terms: D000208:Acute Disease; D000368:Aged; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology / Q000601:surgery*; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000601:surgery; D003937:Diagnosis, Differential; D004211:Disseminated Intravascular Coagulation / Q000209:etiology; D006498:Hepatectomy; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007249:Inflammation / Q000209:etiology*; D008099:Liver / Q000473:pathology; D008297:Male; D018805:Sepsis / Q000209:etiology*; D012720:Severity of Illness Index; D013492:Suppuration / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D007074:Immunoglobulin G