内皮素受体拮抗剂与内皮素受体结合后，可以抑制由内皮素引起的血管收缩，同时还可减轻由内皮素活化造成的血管平滑肌的增殖和纤维化，从而达到治疗与内皮素相关疾病的目的。马西替坦是由瑞士Actelion公司研发的新型内皮素受体拮抗剂，具有组织靶向性，对ETA受体、ETB受体具有双重抑制作用，可用于治疗肺动脉高压、肺纤维化等疾病，在一系列临床研究中显示出良好的治疗前景，且安全性及耐受性均较好。

Endothelin receptor antagonists combined with endothelin receptor could inhibit vasoconstriction induced by endothelin, and also could reduce the proliferation and fibrosis which are caused by the activition of endothelin. Macitentan is a new endothelin receptor antagonist developed by Switzerland Actelion Company, which has the organization target with dual inhibitory effects on ETA and ETB receptor. Macitentan could be used for the treatment of pulmonary arterial hypertension, pulmonary fibrosis, and other diseases, and it shows a good prospect in a series of clinical studies with good safety and tolerability.