原发性胆汁性胆管炎的免疫遗传研究现状

Current status of immunogenetic studies on primary biliary cholangitis

原发性胆汁性胆管炎(PBC)的病理特征是免疫介导的小胆管上皮细胞凋亡性坏死所导致的进行性肝内胆汁淤积,具有进展为胆管纤维化、肝硬化和肝细胞癌的风险。PBC具有免疫遗传特性,PBC患者免疫应答的遗传调控异常包括人类白细胞抗原(HLA)和非HLA基因参与的、以肝内小胆管上皮细胞线粒体内的丙酮酸脱氢酶复合体E2亚基为抗原的T、B淋巴细胞免疫应答异常。超过30%的PBC患者对熊去氧胆酸治疗反应差,因此明确PBC免疫调控异常的机制对PBC免疫治疗具有广泛临床指导意义。

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.