CAJ | 학술논문

内皮素是强的缩血管因子，肺动脉高压患者内皮素表达明显增加，内皮素受体拮抗剂已经作为靶向药物治疗肺动脉高压，其疗效及前景备受瞩目。波生坦、安立生坦分别为口服的双重和选择性内皮素受体拮抗剂（ERAs），均能显著降低肺动脉高压，改善患者的生存， Macitentan是一种新型的口服非肽类双重ERAs，现已完成Ⅲ期临床研究，疗效可观；西他生坦是选择性的内皮素受体拮抗剂，因其肝脏毒性已经退市。对于这些双重或选择性内皮素受体拮抗剂的选择问题至今仍存在分歧，长期疗效、联合用药及肝脏安全性等问题都需要更多循证医学证实。
내피소시강적축혈관인자，폐동맥고압환자내피소표체명현증가，내피소수체길항제이경작위파향약물치료폐동맥고압，기료효급전경비수촉목。파생탄、안립생탄분별위구복적쌍중화선택성내피소수체길항제（ERAs），균능현저강저폐동맥고압，개선환자적생존， Macitentan시일충신형적구복비태류쌍중ERAs，현이완성Ⅲ기림상연구，료효가관；서타생탄시선택성적내피소수체길항제，인기간장독성이경퇴시。대우저사쌍중혹선택성내피소수체길항제적선택문제지금잉존재분기，장기료효、연합용약급간장안전성등문제도수요경다순증의학증실。
Since the endothelin mediates a potent vasoconstrictive response and its expression has been found to increase in patients with pulmonary arterial hypertension(PAH), endothelin receptor antagonists(ERAs) has been emerged as an essential target therapeutic strategy in PAH with a bright future. Both bosentan and ambrisentan, as a dual and a selective ERA, are effective and oral agents for a signiifcant reduction in pulmonary arterial pressure and improvement in survival. Macitentan belongs to an new non-peptide and non-selective ERAs, proved to be efficacious according to its phase III trial. Sitaxsentan is a highly selective oral endothelin-A receptor antagonist, however, liver toxicity led to its withdrawal. It is debated whether selective or dual ET blockade is the best approach for treating PAH. Furthermore, better clinical trials on long-term efficacy evaluations, combination of drugs and consideration of liver safety are needed to guide therapeutic management.