肺动脉高压发病机制中的肺血管重塑  

Pulmonary vascular remodeling in the pathogenesis of pulmonary arterial hypertension

作　　者：张嘉莹 樊勇[1] 张卓莉[1] ZHANG Jia-ying;FAN Yong;ZHANG Zhuo-li(Department of Rheumatology and Clinical Immunology,Peking University First Hospital,Beijing100034,China)

机构地区：[1]北京大学第一医院风湿免疫科,北京100034

出　　处：《中华临床免疫和变态反应杂志》2023年第1期50-55,共6页Chinese Journal of Allergy & Clinical Immunology

摘　　要：肺动脉高压是一类临床危重的心肺血管疾病,以肺血管阻力增加、进行性肺动脉压力升高、最终导致右心衰竭,甚至死亡为临床特点。肺血管重塑在肺动脉高压的发生发展过程中扮演着关键角色,尽管近年来靶向药物治疗已经显著改善了肺动脉高压患者的预后,目前的药物主要靶向血管舒缩功能,同时有部分改善血管重塑的作用,但无法根本上逆转肺血管重塑,故患者预后依然较差。本文从遗传机制、低氧及气体分子机制、炎症和免疫异常机制以及当前的一些新机制(内皮间质转化、异常能量代谢、表观遗传调节)等方面综述肺动脉高压发病机制中肺血管重塑的研究进展,以期为肺动脉高压的治疗提供新的思路。Pulmonary arterial hypertension(PAH)is a clinically critical cardiopulmonary vascular disease characterized by increased pulmonary vascular resistance,progressive pulmonary artery pressure,and eventually leads to right heart failure and even death.Pulmonary vascular remodeling plays a key role in the PAH pathogenesis.Although targeted agents have significantly improved the overall prognosis of patients with PAH by mainly targeting vasomotor function and a partial effect on vascular remodeling,they cannot reverse pulmonary vascular remodeling.Hence,prognosis of PAH patients remains unsatisfied.In order to provide new perspectives for the PAH treatment,current research progress of pulmonary vascular remodeling in the pathogenesis of PAH is compressively reviewed,generally from the aspects of genetic mechanism,hypoxia and gas molecular mechanism,inflammation and immune abnormality mechanism,to some novel mechanisms(endothelial to mesenchymal transition,abnormal energy metabolism,and epigenetic regulation).

关 键 词：肺动脉高压 血管重塑 免疫异常 遗传机制 

分 类 号：R544.1[医药卫生—心血管疾病]