The etiology of primary biliary cirrhosis remains largely unknown despite numerous lines of evidence that have been recently proposed or supported. Primary biliary cirrhosis is a chronic cholestatic liver disease for which an autoimmune pathogenesis is widely accepted, mostly based on the presence of autoantibodies and autoreactive T cells. Cumulatively, association and twin studies suggest that both a susceptible genetic background and environmental factors determine disease onset. Multiple infectious and chemical candidates may contribute to the disease onset in a genetically susceptible host. Several murine models have been recently reported and include genetically determined ones as well as models induced by immunization with chemicals and bacteria.
- Antimitochondrial autoantibodies
- Female predominance
- Primary biliary cirrhosis
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