Open AccessPrimary biliary cirrhosis: What do autoantibodies tell us?
Author(s) -
Chaofan Hu,
Fengchun Zhang,
Yongzhe Li,
Xuan Zhang
Publication year - 2010
Publication title -
world journal of gastroenterology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.427
H-Index - 155
eISSN - 2219-2840
pISSN - 1007-9327
DOI - 10.3748/wjg.v16.i29.3616
Subject(s) - primary biliary cirrhosis , autoantibody , medicine , etiology , differential diagnosis , primary sclerosing cholangitis , serology , fibrosis , autoimmune disease , immunology , autoimmune hepatitis , gastroenterology , pathology , disease , antibody
Primary biliary cirrhosis (PBC) is a chronic, progressive, cholestatic, organ-specific autoimmune disease of unknown etiology. It predominantly affects middle-aged women, and is characterized by autoimmune-mediated destruction of small- and medium-size intrahepatic bile ducts, portal inflammation and progressive scarring, which without proper treatment can ultimately lead to fibrosis and hepatic failure. Serum autoantibodies are crucial tools for differential diagnosis of PBC. While it is currently accepted that antimitochondrial antibodies are the most important serological markers of PBC, during the last five decades more than sixty autoantibodies have been explored in these patients, some of which had previously been thought to be specific for other autoimmune diseases.