Development of novel therapies targeting at dysregulated signaling pathways in pulmonary arterial hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a complex pulmonary vascular disease characterized by progressive elevation of mean pulmonary artery pressure resulted from the pathological feature of pulmonary vascular remodeling. Without medical intervention, PAH can eventually lead to right heart failure and death of patients. Up to the present, there are few treatment options for PAH are still mainly function through pulmonary vasodilation. Although these treatments can alleviate symptoms, the prognosis remains poor. In recent years, breakthroughs have been made in understanding the pathogenesis of PAH, thus support the development of new treatment strategies targeting at dysregulation of signaling pathways in PAH. This review focuses on five critical pathways and the relevant drugs those entered phase Ⅱ clinical trials and discusses their therapeutic potential, so to provide a basis for future research on targeting therapies for PAH patients.

Keywords

• pulmonary arterial hypertension|molecular pathways|ligands and receptors|targeted therapy|drug development