IgG4-related sclerosing cholangitis- A great mimicker

Indian J Gastroenterol. 2020 Dec;39(6):614-618. doi: 10.1007/s12664-020-01102-2. Epub 2020 Nov 6.

Abstract

IgG4-related disease (IgG4 RD) is a multisystem disorder characterized by tumefactive (mass forming) lesions, storiform fibrosis, and IgG4-positive plasma cell infiltration. IgG4 sclerosing cholangitis (IgG4 SC) is the biliary manifestation of IgG4 RD, often in association with autoimmune pancreatitis (AIP). Here, we report two patients with IgG4-related cholangitis mimicking primary sclerosing cholangitis.

Keywords: Autoimmune pancreatitis; Cholestatic jaundice; IgG4 sclerosing cholangitis; Primary sclerosing cholangitis.

MeSH terms

  • Autoimmune Pancreatitis
  • Cholangitis, Sclerosing / diagnosis
  • Cholangitis, Sclerosing / immunology
  • Cholangitis, Sclerosing / pathology
  • Diagnosis, Differential
  • Female
  • Fibrosis
  • Humans
  • Immunoglobulin G4-Related Disease / diagnosis*
  • Immunoglobulin G4-Related Disease / immunology*
  • Immunoglobulin G4-Related Disease / pathology
  • Male