title: Recognition of other organ involvement might assist in the differential diagnosis of IgG4-associated sclerosing cholangitis without apparent pancreatic involvement: report of two cases.
abstract: IgG4-associated sclerosing cholangitis (IAC) was recently defined as biliary involvement of IgG4-related systemic disease. It is frequently associated with autoimmune pancreatitis, characterized by pancreatic enlargement and irregular narrowing of the pancreatic duct. However, a few cases of IAC with no apparent pancreatic involvement have been described, the characteristics of which may mimic those of cholangiocarcinoma. We report two rare cases of IgG4-associated sclerosing cholangitis at the hepatic hilum, mimicking hilar cholangiocarcinoma. When trying to establish the diagnosis, we should consider other organs that could be involved, such as the pancreas, salivary glands, retroperitoneum, lymph nodes, and kidneys, as well as chronic inflammatory changes. By recognizing these lesions and measuring serum IgG4, IAC can be diagnosed correctly, thereby avoiding unnecessary major surgery for a condition that is treated effectively by steroid therapy.
authors.affiliation: Department of Surgery, Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, 305-8575, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nowatari
authors.forename: Akihiko
authors.initials: A
authors.lastname: Kobayashi
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Fukunaga
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Oda
authors.forename: Ryoko
authors.initials: R
authors.lastname: Sasaki
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Ohkohchi
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D001650:Bile Duct Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D002760:Cholangiopancreatography, Endoscopic Retrograde / Q000379:methods; D015209:Cholangitis, Sclerosing / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology* / Q000378:metabolism; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D050500:Pancreatitis, Chronic / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G