Current status of immunogenetic studies on primary biliary cholangitis
10.3969/j.issn.1001-5256.2020.04.050
- VernacularTitle:原发性胆汁性胆管炎的免疫遗传研究现状
- Author:
Weiyu MA
1
;
Zhihua DENG
Author Information
1. Department of Gastroenterology, The Second Hospital of Shanxi Medical University, Taiyuan 030001, China
- Publication Type:Research Article
- Keywords:
primary biliary cholangitis;
gene;
immunity;
HLA antigens
- From:
Journal of Clinical Hepatology
2020;36(4):932-935
- CountryChina
- Language:Chinese
-
Abstract:
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.
