Published online Mar 14, 2014. doi: 10.3748/wjg.v20.i10.2606
Revised: November 22, 2013
Accepted: January 8, 2014
Published online: March 14, 2014
Core tip: While the presence of antimitochondrial antibodies is pathognomonic to Primary biliary cirrhosis (PBC), more than 60 autoantibodies have been detected in patients with PBC. Antinuclear antibodies (ANA) become positive in approximately 30% to 50% of patients with PBC. Among ANA, anti-gp210 and anticentromere antibodies have been indicated as significant prognostic markers. Previous studies have shown that unique characteristics of biliary epithelial cells during apoptosis may result in the presence of a direct and specific immune response to mitochondrial autoantigens. Moreover, recent studies have indicated that chemicals originating from the environment are associated with a breakdown in the tolerance against mitochondrial autoantigens.