Boucherat Olivier, Vitry Geraldine, Trinh Isabelle, Paulin Roxane, Provencher Steeve, Bonnet Sebastien
Pulmonary Hypertension and Vascular Biology Research Group, Institut Universitaire de Cardiologie et de Pneumologie de Québec, Université Laval, Department of Medicine, Québec, Canada.
Pulm Circ. 2017 Apr-Jun;7(2):285-299. doi: 10.1177/2045893217701438. Epub 2017 Mar 27.
Pulmonary arterial hypertension (PAH) remains a mysterious killer that, like cancer, is characterized by tremendous complexity. PAH development occurs under sustained and persistent environmental stress, such as inflammation, shear stress, pseudo-hypoxia, and more. After inducing an initial death of the endothelial cells, these environmental stresses contribute with time to the development of hyper-proliferative and apoptotic resistant clone of cells including pulmonary artery smooth muscle cells, fibroblasts, and even pulmonary artery endothelial cells allowing vascular remodeling and PAH development. Molecularly, these cells exhibit many features common to cancer cells offering the opportunity to exploit therapeutic strategies used in cancer to treat PAH. In this review, we outline the signaling pathways and mechanisms described in cancer that drive PAH cells' survival and proliferation and discuss the therapeutic potential of antineoplastic drugs in PAH.
肺动脉高压（PAH）仍然是一个神秘的杀手，与癌症一样，其特点是极其复杂。PAH的发展发生在持续的环境压力下，如炎症、剪切应力、假性缺氧等。在内皮细胞最初死亡后，随着时间的推移，这些环境压力促使包括肺动脉平滑肌细胞、成纤维细胞甚至肺动脉内皮细胞在内的细胞形成过度增殖且抗凋亡的克隆，从而导致血管重塑和PAH的发展。从分子层面来看，这些细胞表现出许多与癌细胞共有的特征，这为利用癌症治疗策略来治疗PAH提供了机会。在这篇综述中，我们概述了癌症中描述的驱动PAH细胞存活和增殖的信号通路和机制，并讨论了抗肿瘤药物在PAH中的治疗潜力。
