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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。
Arterial pulmonary hypertension (PAH) is a fatal disease characterized by persistent increase of pulmonary artery pressure and persistent structural changes of pulmonary arterioles. Pulmonary remodeling is the main pathological feature. Many studies found that the pathological mechanism of vascular remodeling in PAH mainly includes the pathological changes of various cells and the involvement of multiple molecular signaling pathways. Recent studies indicate that epigenetics is also involved in vascular remodeling, which leads to the development of the disease. Currently, various new drugs and new therapies aimed at the pathogenesis of PAH are being developed with less side effects and better therapeutic effects. This article focuses on the pathogenesis of PAH vascular remodeling, including pathological changes, new molecular signaling pathways and epigenetic content, as well as new advances in treatment.