Dept of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA
Faculté de Médecine, Université Paris-Sud, Université Paris-Saclay, Le Kremlin-Bicêtre, France.
Eur Respir Rev. 2017 Dec 20;26(146). doi: 10.1183/16000617.0093-2017. Print 2017 Dec 31.
The pathobiology of pulmonary arterial hypertension (PAH) is complex and incompletely understood. Although three pathogenic pathways have been relatively well characterised, it is widely accepted that dysfunction in a multitude of other cellular processes is likely to play a critical role in driving the development of PAH. Currently available therapies, which all target one of the three well-characterised pathways, provide significant benefits for patients; however, PAH remains a progressive and ultimately fatal disease. The development of drugs to target alternative pathogenic pathways is, therefore, an attractive proposition and one that may complement existing treatment regimens to improve outcomes for patients. Considerable research has been undertaken to identify the role of the less well-understood pathways and in this review we will highlight some of the key discoveries and the potential for utility as therapeutic targets.
肺动脉高压（PAH）的发病机制复杂且尚未完全阐明。虽然已经相对清楚地描述了三种发病途径，但人们普遍认为，许多其他细胞过程的功能障碍可能在推动 PAH 的发展中起关键作用。目前可用的治疗方法均靶向三种已充分阐明的途径之一，为患者带来了显著益处；然而，PAH 仍然是一种进行性的、最终致命的疾病。因此，开发靶向其他发病途径的药物是一种有吸引力的方案，可能会补充现有的治疗方案，以改善患者的预后。已经进行了大量研究以确定了解较少的途径的作用，在本综述中，我们将重点介绍一些关键发现以及作为治疗靶点的潜力。
