自身免疫性肝病与门静脉血栓的关系概述

Overview of the Relationship between Autoimmune Liver Disease and Portal Vein Thrombosis

摘要: 自身免疫性肝病(Autoimmune liver disease, AiLD)是一组以肝脏免疫介导损伤为特征的慢性疾病，主要包括自身免疫性肝炎(Autoimmune hepatitis, AIH)、原发性胆汁性胆管炎(Primary biliary cirrosis, PBC)、原发性硬化性胆管炎(Primary sclerosing cholangitis, PSC)等；目前关于该组疾病的发病机制尚未明确，若未及时处理，严重可致患者死亡。门静脉血栓(Portal vein thrombosis, PVT)作为AiLD患者的严重并发症，可使腹水加重、出血等不良事件的概率增加，PVT的发生与多种病因有关，包括门静脉高压(portal hypertention, PH)、高凝状态、血管内皮损伤等；AiLD作为自身免疫介导的疾病，既往有研究提出AIH、PBC等可能在非肝硬化阶段就已经存在血液的高凝状态，继而发生PVT，因此，本文将对既往关于二者关系的文献报道进行叙述性综述。

Abstract: Autoimmune liver disease (AiLD) is a group of chronic diseases characterized by immune-mediated liver injury, mainly including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), etc. The pathogenesis of this group of diseases has not been fully clarified, and severe cases can lead to death if not treated in a timely manner. Portal vein thrombosis (PVT) is a serious complication of AiLD, which can increase the risk of adverse events such as aggravated ascites and bleeding. The occurrence of PVT is related to multiple etiologies, including portal hypertension (PH), hypercoagulable state, vascular endothelial injury, etc. As an autoimmune-mediated disease, previous studies have suggested that AIH, PBC and other AiLD may already have a hypercoagulable state in the non-cirrhotic stage, which in turn leads to PVT. Therefore, this paper presents a narrative review of existing literature on the relationship between AiLD and PVT.