Objective To compare the curative effect and tolerability of 4 kinds of endothelin receptor antagonists (ambrisentan, bosentan, sitaxsentan and macitentan) in treatment of pulmonary arterial hypertension (PAH) by applying network Meta-analysis.Methods The databases of PubMed, EMBase, Cochrane Library, CBM, CNKI and WanFang Database were retrieved with computer until Jan. 31, 2014 for collecting randomized controlled trials (RCT) about treatment of PAH with 4 kinds of endothelin receptor antagonists. The primary outcome indexes were curative effect and tolerability and secondary ones were mortality and clinical worsening rate. All data was screened, extracted and reviewed by 2 researchers independently, compared directly and given network Meta-analyzed by using RevMan5.2 software and GEMTC software.Results There were 12 documents enclosed including 13 RCT involving 2191 patients. The direct Meta-analysis showed that the curative effect of 4 kinds of endothelin receptor antagonists was all better than that of placebo, and tolerability of only ambrisentan was better than that of placebo. The network Meta-analysis showed that the curative effect of ambrisentan and bosentan was better than that of placebo. The rank probability plot showed that the curative effect of ambrisentan was the best and then bosentan, sitaxsentan and macitentan in a descending order. The tolerability of ambrisentan and sitaxsentan were better than that of placebo. The rank probability plot showed that the tolerability of ambrisentan was the highest and then sitaxsentan, macitentan and bosentan in a descending order.Conclusion Among 4 kinds of endothelin receptor antagonists, ambrisentan is superior to other 3 in curative and tolerability. In the future studies, the effects of ambrisentan and other drugs on mortality and clinical worsening rate should be paid more attention to. In addition the curative effects of different drug dosage and specific effect on different types of PAH patients should be investigated.%目的：采用网状Meta分析的方法比较安贝生坦、波生坦、西他生坦和马西替坦四种内皮素受体拮抗剂治疗肺动脉高压的疗效和耐受性。方法计算机检索PubMed、EMBASE、Cochrane Library和中国生物医学文献数据库、中国知网、万方数据库，检索时间截至2014年1月31日，纳入比较四种内皮素受体拮抗剂治疗肺动脉高压的随机对照试验，主要结局指标为疗效和耐受性，次要结局指标为病死率和临床恶化率。研究筛选、数据提取及方法学质量评价均由两人独立完成。分别使用 RevMan 5.2和GEMTC软件进行直接比较和网状Meta分析。结果纳入12篇文献，包含13个随机对照试验，总计2191例患者。直接Meta分析结果显示，4种内皮素受体拮抗剂疗效均优于安慰剂，在耐受性方面，只有安贝生坦的效果优于安慰剂，其余药物与安慰剂的比较差异无统计学意义。网状Meta分析结果显示：在疗效方面，安贝生坦和波生坦的疗效优于安慰剂，西他生坦及马西替坦与安慰剂比较以及各种药物的互相比较差异均无统计学意义。等级概率图显示：安贝生坦效果最佳，其次为波生坦、西他生坦，马西替坦疗效最差。在耐受性方面，安贝生坦与西他生坦优于安慰剂，其余药物与安慰剂的比较以及各类药物之间的比较差异均无统计学意义。等级概率图显示：耐受性最好的药物为安贝生坦，其余依次为西他生坦、马西替坦，最差为波生坦。结论在治疗肺动脉高压的4种内皮素受体拮抗剂中，安贝生坦在疗效和耐受性方面均优于其他3种药物。在未来的研究中，应该更多关注安贝生坦及其他药物在病死率和临床恶化率方面的效果，此外，应注重探讨不同药物剂量、不同类型肺动脉高压患者的具体疗效。
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