血管重构在动脉型肺动脉高压中的研究进展

动脉型肺动脉高压（PAH）是以肺动脉压力持续升高、肺小动脉结构持续改变为特征的致命性疾病，肺动脉重构是其主要的病理学特点。许多研究发现PAH中血管重构的病理机制主要包括各种细胞的病理学改变和多种分子信号通路的参与，最新的研究指出表观遗传学也参与血管重构，从而导致疾病的发生发展。目前针对PAH发病机制的各种新型药物及新疗法正在被开发，其副作用更小、疗效更佳。本文重点阐述PAH中血管重构的病理机制包括病理学改变、新分子信号通路及表观遗传学内容，以及治疗的新进展。

Advances in vascular remodeling of pulmonary arterial hypertension

Pulmonary arterial hypertension(PAH) is a fatal disease characterized by a persistent elevation of pulmonary arterial pressure and persistent structural changes in small pulmonary arterioles. The main pathological feature is vascular remodeling of the pulmonary arteries. Many studies have suggested that its pathologic mechanism includes changes of various cells and several molecular signaling pathways. Recent research indicates that vascular remodeling also involves epigenetic components, resulting in the development of PAH. Various novel therapies targeting the pathological mechanism are being developed and designed to have fewer side effects and higher efficacy. This review focuses on the pathological mechanism including pathological changes, novel signaling pathways, and epigenetics as well as advances in treatment of PAH.