The etiology mystery in primary biliary cirrhosis

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.