Pulmonary arterial hypertension (PAH) is a frequent but severe vascular complication of patients with connective tissue diseases (CTDs) and a major cause of significant morbidity and mortality in these patients.

Over the past few decades, effective therapies that targeting key signaling pathways involved in PAH have significantly improved patients symptoms and quality of life, and CTD-PAH patients are also greatly benefit from them. However, the current treatments fail to be completely curative, and prognosis of PAH patients remains poor. On the other hand, the role of inflammation underlying the pathogenesis of CTD-PAH should be emphasized, considering the better clinical effectiveness of immunosuppressive therapy for CTD-PAH patients. Meanwhile, there are more research progresses, novel therapeutic strategies, and updated clinical concepts, including the pivotal role of immunosuppressive therapy, treatment goals of “dual treat-to-target”, in the field of CTD-PAH. Therefore, this article will discuss the possible pathogenesis, treatment strategies, and promising therapeutic interventions in CTD-PAH.

肺动脉高压 (PAH) 是结缔组织病 (CTD) 患者常见但严重的血管并发症，也是这些患者发病率和死亡率显着升高的主要原因。

在过去的几十年里，针对PAH相关关键信号通路的有效疗法显着改善了患者的症状和生活质量，CTD-PAH患者也从中受益匪浅。然而，目前的治疗方法未能完全治愈，肺动脉高压患者的预后仍然很差。另一方面，应强调炎症在 CTD-PAH 发病机制中的作用，考虑到 CTD-PAH 患者免疫抑制治疗的临床效果更好。同时，在CTD-PAH领域也有更多的研究进展、新的治疗策略和更新的临床理念，包括免疫抑制治疗的关键作用、“双重治疗达标”的治疗目标。因此，本文将探讨可能的发病机制、治疗策略、