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

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

Differential Diagnosis

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Cases and figures

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