Division of Pulmonary Critical Care Medicine, University of New Mexico, Albuquerque, New Mexico, USA.
Curr Opin Pulm Med. 2022 Sep 1;28(5):361-368. doi: 10.1097/MCP.0000000000000887. Epub 2022 Jul 16.
With the establishment of vasodilator therapy as a mainstay of treatment for pulmonary arterial hypertension (PAH), new therapeutic approaches are needed to prevent the development of the vasculopathy associated with this disease. Many studies are currently underway to investigate nonvasodilator treatment options.
Modulation of bone morphogenic protein receptor type 2 (BMPR2) signaling with sotatercept showed promising results in phase 2 studies. Rituximab, an anti-CD20 monoclonal antibody, showed some signal for beneficial effect in patients with scleroderma-associated PAH. Studies evaluating agents including tocilizumab, selonsertib, bardoxolone, 10-nitro-9(E)-enoic acid (CXA-10) and intravenous iron have not shown acceptable efficacy in treating PAH.
Pharmacologic approaches for the treatment of PAH include altering of transforming growth factor β/BMPR2 signaling, proliferation via growth factors, immune response, oxidative stress, estrogen signaling, metabolism, and neurohormonal modulation. Other treatment modalities including pulmonary artery nerve denervation, stem cell therapy, and inter-atrial shunt formation are also being explored.
随着血管扩张剂治疗作为肺动脉高压（PAH）治疗的主要方法的确立，需要新的治疗方法来预防与这种疾病相关的血管病变。目前正在进行许多研究以探讨非血管扩张剂治疗选择。
索他洛尔对骨形态发生蛋白受体 2（BMPR2）信号的调节在 2 期研究中显示出有前景的结果。利妥昔单抗，一种抗 CD20 单克隆抗体，在硬皮病相关 PAH 患者中显示出一些有益作用的信号。评估包括托珠单抗、selonsertib、bardoxolone、10-硝基-9（E）-烯酸（CXA-10）和静脉铁在内的药物的研究在治疗 PAH 方面没有显示出可接受的疗效。
PAH 的治疗药物方法包括改变转化生长因子β/BMPR2 信号、生长因子增殖、免疫反应、氧化应激、雌激素信号、代谢和神经激素调节。其他治疗方式包括肺动脉神经去神经支配、干细胞治疗和房间隔分流形成也正在探索中。
