IgG4-related Disease and the Liver

Gastroenterol Clin North Am. 2017 Jun;46(2):195-216. doi: 10.1016/j.gtc.2017.01.001.

Abstract

Pathologists are likely to encounter IgG4-related disease in several organ systems. This article focuses on helping pathologists diagnose IgG4-related disease in the hepatobiliary system. Missing the diagnosis can result in unnecessary organ damage and/or unnecessary surgical and cancer therapy. In the liver, tumefactive lesion(s) involving the bile ducts with storiform fibrosis and an IgG4-enriched lymphoplasmacytic infiltrate are highly concerning for IgG4-related disease. The recent identification of oligoclonal populations of T cells and B cells in IgG4-related disease may lead to molecular tests, new therapeutics, and a greater mechanistic understanding of the disease.

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

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / epidemiology
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / pathology*
  • Biopsy
  • Cholangitis, Sclerosing / diagnosis
  • Cholangitis, Sclerosing / immunology
  • Cholangitis, Sclerosing / pathology*
  • Diagnosis, Differential
  • Granulomatosis with Polyangiitis / diagnosis
  • Humans
  • Immunoglobulin G / analysis*
  • Immunoglobulin G / blood
  • Liver Diseases / diagnosis
  • Liver Diseases / epidemiology
  • Liver Diseases / immunology*
  • Liver Diseases / pathology*
  • Liver Neoplasms / diagnosis
  • Liver Neoplasms / pathology

Substances

  • Immunoglobulin G