title: IgG4-related cholangitis: Case report and literature review.
abstract: METHODS
  We describe a case of a patient who presents with jaundice, elevated cholestatic liver enzymes, an extreme weight loss and a midcholedochal stricture very suspect for a cholangiocarcinoma. In the conviction of malignancy, although the absence of anatomopathological prove, the patient underwent a choledochal resection. The anatomopathological specimen revealed no malignancy. In the year following resection, the patient keeps presenting with bile duct strictures and further weight loss. Ultimately the diagnosis of Ig G4-related cholangitis is withheld. Therapy with corticosteroids is initiated with a spectacular clinical, biochemical and radiographical result.
  
  
  CONCLUSIONS
  IgG4-related cholangitis is the biliary presentation of IgG4-related disease, a recently discovered entity of fibroinflammatory masses which can affect virtually every organ in the body. It is characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, obliterative phlebitis and a presence of > 30 IgG4-positive plasma cells per high power field. Main differential diagnosis contains cholangiocarcinoma and primary sclerosing cholangitis. Corticoids are cornerstone of therapy, with azathioprine frequently used as a maintenance in case of relapse.
  
  
  CONCLUSIONS
  With this case we want to draw the attention to a rather uncommon cause of biliary obstruction, easily mistaken for a cholangiocarcinoma.
authors.forename: A
authors.initials: A
authors.lastname: De Both
authors.forename: H
authors.initials: H
authors.lastname: Van Vlierberghe
authors.forename: A
authors.initials: A
authors.lastname: Geerts
authors.forename: L
authors.initials: L
authors.lastname: Libbrecht
authors.forename: X
authors.initials: X
authors.lastname: Verhelst
mesh_terms: D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D001649:Bile Duct Diseases / Q000175:diagnosis* / Q000209:etiology / Q000276:immunology; D001650:Bile Duct Neoplasms / Q000175:diagnosis*; D018281:Cholangiocarcinoma / Q000175:diagnosis*; D002761:Cholangitis / Q000150:complications / Q000175:diagnosis* / Q000276:immunology; D015209:Cholangitis, Sclerosing / Q000175:diagnosis*; D003251:Constriction, Pathologic / Q000175:diagnosis / Q000209:etiology / Q000276:immunology; D003937:Diagnosis, Differential; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D041781:Jaundice, Obstructive / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010950:Plasma Cells / Q000276:immunology
chemical_list: D007074:Immunoglobulin G