IgG4-related sclerosing cholangitis

Dr Henry Knipe et al.

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

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Article information

rID: 71036
Tag: refs, cases
Synonyms or Alternate Spellings:
  • Autoimmune cholangiopathy
  • IgG4-related sclerosing cholangitis (ISC)

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Cases and figures

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