Immunoglobulin G4-related lymphoplasmacytic sclerosing cholangitis that mimics infiltrating hilar cholangiocarcinoma: part of a spectrum of autoimmune pancreatitis?

Gastrointest Endosc. 2005 Jul;62(1):152-7. doi: 10.1016/s0016-5107(05)00561-4.


Background: Autoimmune pancreatitis has been designated as sclerosing pancreatocholangitis, because this disease shows a high prevalence of bile-duct lesions. We present herein the clinical characteristics of unusual cases that show dominant bile-duct lesions and mimicking infiltrating hilar cholangiocarcinomas.

Methods: Clinical and pathologic findings of 3 patients with immunoglobulin (Ig) G4 related sclerosing cholangitis who had no apparent pancreatic lesions comparable with autoimmune pancreatitis were analyzed.

Observations: All patients were middle-aged or elderly individuals with slightly elevated serum IgG4 concentrations and showed long-segment narrowing of the bile-duct system, mimicking infiltrating hilar cholangiocarcinoma without significant pancreatic change. The first patient was treated with a corticosteroid, resulting in amelioration of the narrowing of the bile duct. The second patient underwent surgery based on a diagnosis of cholangiocarcinoma. In the third patient, the bile-duct stricture reversed spontaneously 1 month after the drainage procedure. Pathologic findings of the bile ducts for all patients disclosed significant lymphoplasmacytic infiltration, including abundant IgG4-bearing plasma cells.

Conclusions: The use of IgG4 immunostaining in biopsy specimens of the bile duct may identify the presence of corticosteroid-responsive lymphoplasmacytic sclerosing cholangitis.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Antibodies, Anti-Idiotypic / immunology*
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / immunology*
  • Bile Duct Neoplasms / diagnosis*
  • Bile Ducts, Intrahepatic* / diagnostic imaging
  • Bile Ducts, Intrahepatic* / pathology
  • Biopsy
  • Cholangiocarcinoma / diagnosis*
  • Cholangiopancreatography, Endoscopic Retrograde
  • Cholangitis, Sclerosing / diagnosis
  • Cholangitis, Sclerosing / immunology*
  • Diagnosis, Differential
  • Female
  • Fluorescent Antibody Technique, Indirect
  • Follow-Up Studies
  • Humans
  • Immunoglobulin G / immunology*
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Radionuclide Imaging


  • Antibodies, Anti-Idiotypic
  • Immunoglobulin G