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World J Gastroenterol. Mar 14, 2014; 20(10): 2606-2612
Published online Mar 14, 2014. doi: 10.3748/wjg.v20.i10.2606
Autoantibodies in primary biliary cirrhosis: Recent progress in research on the pathogenetic and clinical significance
Satoshi Yamagiwa, Hiroteru Kamimura, Masaaki Takamura, Yutaka Aoyagi
Satoshi Yamagiwa, Hiroteru Kamimura, Masaaki Takamura, Yutaka Aoyagi, Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata 951-8510, Japan
Author contributions: All authors contributed to the analysis of pertinent literature and to the writing of the manuscript.
Correspondence to: Satoshi Yamagiwa, MD, PhD, Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Sciences, 757 Asahimachi-dori 1, Chuo-ku, Niigata 951-8510, Japan. syamagi@med.niigata-u.ac.jp
Telephone: +81-25-2272207 Fax: +81-25-2270776
Received: October 26, 2013
Revised: November 22, 2013
Accepted: January 8, 2014
Published online: March 14, 2014
Processing time: 136 Days and 15.7 Hours
Core Tip

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.