New Thoughts on Immunoglobulin G4-Related Sclerosing Cholangitis

Clin Liver Dis. 2016 Feb;20(1):47-65. doi: 10.1016/j.cld.2015.08.004. Epub 2015 Oct 6.


Immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of the multisystem IgG4-related disease. IgG4-SC presents with biliary strictures and/or masses that can bear a striking similarity to other malignant and inflammatory diseases. Diagnosis is based on a combination of clinical, biochemical, radiological, and histologic findings with careful exclusion of malignant disease. Corticosteroids are the mainstay of treatment with good clinical, biochemical, and radiological responses. This review provides a comprehensive overview of the current knowledge of the prevalence, clinical features, radiology and histology findings, diagnosis, treatment, natural history, and pathophysiology of IgG4-SC.

Keywords: Autoimmune pancreatitis; IgG4-associated cholangitis; IgG4-related disease; IgG4-related sclerosing cholangitis.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology*
  • Chemokines / metabolism
  • Cholangitis, Sclerosing / diagnosis*
  • Cholangitis, Sclerosing / drug therapy
  • Cholangitis, Sclerosing / immunology*
  • Humans
  • Immunoglobulin G / immunology*
  • Prognosis
  • T-Lymphocytes, Helper-Inducer / immunology
  • T-Lymphocytes, Regulatory / immunology


  • Chemokines
  • Immunoglobulin G