IgG4-Related Sclerosing Cholangitis: A Clinical and Imaging Review

AJR Am J Roentgenol. 2019 Dec;213(6):1221-1231. doi: 10.2214/AJR.19.21519. Epub 2019 Sep 11.


OBJECTIVE. The purpose of this article is to present the pathologic and clinical features of IgG4-related sclerosing cholangitis (ISC), illustrate the associated imaging findings, and discuss treatment of the disorder. CONCLUSION. ISC is an inflammatory disorder involving the biliary system and resulting in strictures. Although often associated with autoimmune pancreatitis, it may be an isolated disease. Differentiation of ISC from other forms of cholangitis and cholangiocarcinoma is difficult but necessary for management. Imaging is important in diagnosing and assessing the extent of disease and planning a management strategy.

Keywords: IgG4-related disease; IgG4-related sclerosing cholangitis; autoimmune pancreatitis; primary sclerosing cholangitis; sclerosing cholangitis.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / diagnostic imaging
  • Bile Duct Neoplasms / diagnostic imaging
  • Bile Ducts, Intrahepatic / diagnostic imaging
  • Cholangiocarcinoma / diagnostic imaging
  • Cholangitis, Sclerosing / diagnostic imaging*
  • Cholangitis, Sclerosing / pathology
  • Diagnosis, Differential
  • Humans
  • Immunoglobulin G*
  • Pancreatitis / diagnostic imaging


  • Immunoglobulin G