原发性胆汁性胆管炎免疫发病机制

Immune pathogenesis of primary biliary cholangitis

原发性胆汁性胆管炎(PBC)是一种以慢性进行性胆汁淤积为主要特征的自身免疫性肝病,针对自身抗原——丙酮酸脱氢酶复合体E2亚单位(PDC-E2)免疫耐受性的丧失是PBC发病的根源。肝内胆管上皮细胞特有的免疫生物学特性使其成为PBC发病的积极参与者。近年来,PBC的检出率逐年提高,但临床上仍未改变熊去氧胆酸单一用药的格局,深入了解PBC的免疫发病机制将有助于临床医师更好地预防和治疗疾病。

Primary biliary cholangitis(PBC)is an autoimmune liver disease,mainly characterized by chronic progressive cholestasis.The root cause of PBC is the loss of immune tolerance to autoantigen E2 subunit of pyruvate dehydrogenase(PDC-E2).The unique immunobiological characteristics of intrahepatic bile duct epithelial cells make it an active participant in the pathogenesis of PBC.In recent years,the detection rate of PBC has been increasing year by year,but the clinical situation of ursodeoxycholic acid monotherapy has not changed.Therefore,an in-depth understanding of the immune pathogenesis of PBC will help clinicians better prevent and treat diseases.