中文English
ISSN 1001-5256 (Print)
ISSN 2097-3497 (Online)
CN 22-1108/R
Volume 36 Issue 4
Apr.  2020
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Article Contents

Current status of immunogenetic studies on primary biliary cholangitis

DOI: 10.3969/j.issn.1001-5256.2020.04.050
  • Published Date: 2020-04-20
  • Primary biliary cholangitis( PBC) has the pathological feature of progressive intrahepatic cholestasis caused by immune-mediated apoptotic necrosis of small biliary epithelial cells,with a risk of progression to bile duct fibrosis,liver cirrhosis,and hepatocellular carcinoma. PBC has immunogenetic characteristics,and the abnormal genetic regulation of immune response in patients with PBC includes abnormal immune response of T and B lymphocytes involving human leukocyte antigen( HLA) and non-HLA genes and taking pyruvate dehydrogenase complex-E2 in mitochondria of intrahepatic small biliary epithelial cells as the antigen. More than 30% of PBC patients have poor response to ursodeoxycholic acid treatment,and therefore,clarifying the mechanism of abnormal immune regulation in PBC has great clinical significance in guiding the immunotherapy for PBC.

     

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