The treatment strategy of connective tissue disease associated pulmonary arterial hypertension: Evolving into the future

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.