Abstract: OBJECTIVE To systematically review low-dose of azithromycin combined with simvastatin in treatment of chronic obstructive pulmonary disease(COPD) combined with pulmonary hypertension(PH). METHODS Databases including PubMed, Medline, CNKI, VIP and WanFang Data were searched to collect randomized controlled trials (RCTs) about low-dose of azithromycin combined with simvastatin in the treatment of COPD combined with PH from Jan. 2000 to Oct. 2016. Two reviewers independently screened literature, extracted data and assessed the risk of bias of included studies. Then meta-analysis was conducted by RevMan 5.2 software. RESULTS Total of 11 RCTs, 943 patients were included. Meta-analysis showed that, low-dose of azithromycin combined with simvastatin could significantly improve the COPD combined with PH patients in FEV1MD=0.25, 95%CI(0.18, 0.32), P>0.001, SaO₂%MD=3.89, 95%CI(3.29, 4.50), P>0.001, 6MWDMD=76.25, 95%CI(71.95, 80.55, P>0.01, meanwhile significantly reduce in pulmonary hypertensionMD=-1.54, 95%CI(-1.83, -1.24), P>0.001 and CRPMD=-8.86, 95%CI(-10.49, -7.23), P>0.001. There were no serious adverse reactions in both groups. The inverted funnel plot suggested that the inclusion of the research publication bias was small. CONCLUSION Low-dose of azithromycin combined with simvastatin can improve lung function, dyspnea, the activity tolerancen and reduce pulmonary hypertension in patients with COPD combined with PH.