Abstract: Primary biliary cirrhosis (PBC) is a chronic and slowly progressive
cholestatic liver disease of autoimmune etiology, characterized by injury of
the intrahepatic bile ducts that may eventually lead to cirrhosis and liver
failure. Evidence suggests cardinal roles for both environmental factors and
genetic susceptibility. Nevertheless, the absolute etiology of PBC is unclear,
despite recent well-designed case-control studies that reported environmental
risk factors, including infectious agents, for PBC. Of the reported infectious
agents, some of them are not reproducible and remain controversial. However,
infection is no doubt one of the major risks among the environmental factors.
This is supported by the fact that infectious agents in autoimmune diseases
express antigens resulting in molecular mimicry and xenobiotics that play a
role in breaking tolerance. Taken together, recent findings from genome wide
assays as well as novel animal models may enable us to better understand the
mechanism of pathogenesis responsible for this disease.
