PMID: 11918275Mar 29, 2002Paper

Primary biliary cirrhosis: lessons learned from an organ-specific disease

Clinical and Experimental Medicine
A NishioM E Gershwin

Abstract

Primary biliary cirrhosis is an autoimmune liver disease that predominantly affects women and is characterized by chronic progressive destruction of small intrahepatic bile ducts with portal inflammation and subsequent fibrosis. The serological hallmark is the presence of antimitochondrial antibodies, which are found in 95% of patients. These antibodies are directed against the 2-oxo-acid dehydrogenase complexes located on the inner membrane of mitochondria. Although the role of antimitochondrial antibodies in the pathogenesis is unknown, the presence of antibodies has allowed detailed immunological definition of the antigenic epitopes, the autoantibodies, and the T-cell response. Theories have been proposed regarding the mechanism of immune-mediated bile duct damage in primary biliary cirrhosis, including the possible role of T-cell-mediated cytotoxicity and molecular mimicry. Primary biliary cirrhosis is usually diagnosed based on the triad of elevated alkaline phosphatase, antimitochondrial antibodies, and characteristic histological changes on liver biopsy. Biochemical liver abnormalities are consistent with the presence of cholestasis and include an elevation of both serum alkaline phosphatase and gamma-glutamyl transpepti...Continue Reading

Citations

Nov 24, 2016·The American Journal of Pathology·Rakesh H BasavalingappaJay Reddy
Mar 17, 2010·Cell Biochemistry and Function·Luisa GenneroAntonio Ponzetto
Jan 11, 2003·Biotechnology & Genetic Engineering Reviews·Antonio D Molina-García
Sep 17, 2003·Journal of Gastroenterology and Hepatology·J J Feld, E J Heathcote
May 7, 2004·The Journal of Immunology : Official Journal of the American Association of Immunologists·Katsushi AmanoM Eric Gershwin

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