title: IgG4-Related Sclerosing Cholangitis.
abstract: More men than women develop immunoglobulin G4-related sclerosing cholangitis (IgG4-SC). Age at clinical onset is significantly older in patients with IgG4-SC. Patients with IgG4-SC appear similar to those with cholangiocarcinoma and primary sclerosing cholangitis (PSC). The association between IgG4-SC and autoimmune pancreatitis (AIP) is useful for the diagnosis of IgG4-SC. However, some IgG4-SC cases are isolated from AIP and are difficult to diagnose. The authors focus on three distinct features of IgG4-SC. First, diffuse inflammation induces a longer stenosis on cholangiography in contrast to the short stenosis of patients with PSC. Second, fibroinflammatory involvement is observed mainly in the stroma of the bile duct wall, whereas the bile duct epithelium is intact. Third, steroid therapy results in remarkable improvement. Although the prognosis of patients with IgG4-SC is good, some cases have developed portal hypertension and liver cirrhosis during their clinical course. Further study is needed to elucidate the long-term outcomes and mechanism of IgG4-SC.
authors.affiliation: Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Nakazawa
authors.affiliation: Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan.
authors.forename: Shuya
authors.initials: S
authors.lastname: Shimizu
authors.affiliation: Department of Gastroenterology and Metabolism, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.
authors.forename: Itaru
authors.initials: I
authors.lastname: Naitoh
mesh_terms: D017668:Age of Onset; D001327:Autoimmune Diseases / Q000175:diagnosis; D001706:Biopsy; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology*; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D010195:Pancreatitis / Q000276:immunology; D013256:Steroids / Q000627:therapeutic use; D014463:Ultrasonography
chemical_list: D007074:Immunoglobulin G; D013256:Steroids