Department of Respiratory Medicine, G. Papanikolaou Hospital , Thessaloniki, Greece.
Department of Respiratory Failure, Aristotle University of Thessaloniki , Thessaloniki, Greece.
Expert Opin Pharmacother. 2020 Jul;21(10):1145-1155. doi: 10.1080/14656566.2020.1727446. Epub 2020 Feb 23.
Pulmonary arterial hypertension (PAH) is still a chronic disorder characterized by high morbidity and mortality. Chronic thromboembolic pulmonary hypertension (CTEPH) is another form of pulmonary hypertension (PH) for which pulmonary endarterectomy (PEA) is the treatment of choice. However, not all patients are operable, while PH is often recurrent or persistent. Thus, for both disorders novel treatment options are urgently needed.
This review describes the mechanism of action of riociguat, a soluble guanylate cyclase (sGC) stimulator, with a dual mode of action. The most relevant publications are presented regarding the efficacy of riociguat in PAH and CTEPH, and also data regarding its potential effect on other forms of PH.
Riociguat is a first-in-class drug approved for the treatment of PAH as a monotherapy or added to endothelin-receptor antagonists as a sequential combination therapy, and for the treatment of inoperable CTEPH or persistent/recurrent PH after PEA. As it can stimulate sGC independently of NO, it could be beneficial in PAH patients with inadequate response to phosphodiesterase 5 inhibitors (PDE5i). Future studies are needed to evaluate whether drug switching is beneficial in PAH and which baseline markers could guide the optimal initial treatment selection.
肺动脉高压（PAH）仍然是一种以高发病率和死亡率为特征的慢性疾病。慢性血栓栓塞性肺动脉高压（CTEPH）是另一种形式的肺动脉高压（PH），肺血管内膜切除术（PEA）是其首选治疗方法。然而，并非所有患者都适合手术，而 PH 往往会复发或持续存在。因此，这两种疾病都迫切需要新的治疗选择。
本文描述了可溶性鸟苷酸环化酶（sGC）刺激剂利奥西呱的作用机制，它具有双重作用模式。本文介绍了关于利奥西呱在 PAH 和 CTEPH 中的疗效的最相关文献，以及关于其对其他形式 PH 的潜在影响的数据。
利奥西呱是一种首创药物，获批用于治疗 PAH，可作为单药治疗，也可作为依前列醇受体拮抗剂的序贯联合治疗，还可用于治疗不能手术的 CTEPH 或 PEA 后持续/复发 PH。由于它可以独立于一氧化氮刺激 sGC，因此对磷酸二酯酶 5 抑制剂（PDE5i）反应不足的 PAH 患者可能有益。需要进一步研究来评估药物转换是否对 PAH 有益，以及哪些基线标志物可以指导最佳初始治疗选择。
