Primary biliary cirrhosis: what do autoantibodies tell us?
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.
Hu
Chao-Jun
CJ
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, No. 41 Da Mu Cang, Western District, Beijing, China.
Zhang
Feng-Chun
FC
Li
Yong-Zhe
YZ
Zhang
Xuan
X
D000818:Animals; D000974:Antibodies, Antinuclear / Q000276:immunology; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology*; D015415:Biomarkers / Q000378:metabolism; D002467:Cell Nucleus / Q000276:immunology / Q000648:ultrastructure; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000097:blood* / Q000276:immunology*; D008928:Mitochondria / Q000276:immunology
D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D015415:Biomarkers