IgG4-related sclerosing cholangitis

Last revised by Mohamed Saber on 8 Jan 2021

IgG4-related sclerosing cholangitis, also known as autoimmune cholangiopathy, is part of the spectrum of IgG4-related disease but can also occur in isolation. It is a separate entity to primary sclerosing cholangitis


The pathogenesis is poorly understood. The disease is characterized by dense bile duct infiltration by IgG4-positive plasma cells resulting in extensive fibrosis. There are often elevated serum IgG4 levels. 


Differential Diagnosis

ADVERTISEMENT: Supporters see fewer/no ads

Cases and figures

  • Case 1
    Drag here to reorder.
  • Case 2
    Drag here to reorder.
  • Case 3
    Drag here to reorder.
  • Updating… Please wait.

     Unable to process the form. Check for errors and try again.

     Thank you for updating your details.