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.
Pathology
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.
Associations
- type 1 autoimmune pancreatitis (90%)
Differential Diagnosis
-
primary sclerosing cholangitis
- associated with inflammatory bowel disease
- younger patients
- less acute, longer duration of symptoms
- does not respond to steroids
- tends to produce shorter segments of stricturing ("beaded")
-
cholangiocarcinoma
- confined to the bile ducts; IgG4 may have other manifestations of disease such as autoimmune pancreatitis
- secondary sclerosing cholangitis
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