Primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome

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Primary biliary cholangitis/primary sclerosing cholangitis and autoimmune hepatitis overlap syndrome

Synonyms: Overlap syndromes of autoimmune liver diseases | PBC/PSC and AIH overlap syndrome

A rare hepatic disease characterized by the overlap of primary biliary cholangitis and/or primary sclerosing cholangitis with autoimmune hepatitis defined by the presence of at least two of the three recognized biochemical serological and histological criteria of each disease. The onset of the overlapping diseases can be simultaneous or sequential with a variable interval of up to several years. Age of onset gender predisposition and clinical phenotype vary between each of the diseases and the clinical presentation ranges from asymptomatic disease or unspecific symptoms such as fatigue arthralgia and pruritus to established cirrhosis and decompensation or also acute fulminant hepatitis and liver failure. Association with extrahepatic autoimmune diseases is common.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version April 2024

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Advocacy Organizations

PSC Partners

The mission of PSC Partners Seeking a Cure is to drive research to identify treatments and a cure for primary sclerosing cholangitis (PSC), while providing education and support for those impacted by this rare disease. We are a U.S.-based nonprofit with global reach via our international research grants, collaborations, international patient registry, and support of the of the PSC community.

Clinical Trials

For a list of clinical trials in this disease area, please click here.