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目的:系统性评价吡非尼酮联合N-乙酰半胱氨酸对于特发性肺纤维化(IPF)的疗效及不良反应。方法:在PubMed、WOS、CNKI、万方、维普检索以吡非尼酮联合N-乙酰半胱氨酸为干预措施治疗IPF的随机对照试验。按Cochrane系统评价方法，评价纳入研究的方法学质量并进行统计分析，采用RevMan5.3进行meta分析。结果:最终纳入8篇文献，合计504例病例。meta分析结果显示，试验组与对照组比较肺功能主要指标用力肺活量的改善具有统计学意义(n SMD＝0.94，95%n CI：0.74～1.13，n P<0.001)，次要指标第1秒用力呼气容积改变也具有统计学意义(n SMD＝1.06，95%n CI：0.83～1.29，n P<0.001)；试验组与对照组不良反应发生率差异无统计学意义(n OR＝0.97，95%n CI：0.54～1.74，n P＝0.91)。n 结论:吡非尼酮联合N-乙酰半胱氨酸比单药治疗特发性肺纤维化更有效，延缓疾病发展进程，且不会增加不良反应的发生率。“,”Objective:To assess the efficacy and adverse reactions of pirfenidone combined with N-acetylcysteine in patients with idiopathic pulmonary fibrosis (IPF) by meta-analysis.Methods:Randomized controlled trials using pirfenidone combined with N-acetylcysteine as an intervention to treat IPF were searched in PubMed, WOS, CNKI, Wanfang and VIP database.Methodological quality evaluation and statistical analysis were conducted by using Review Manager 5.3 according to the Cochrane Collaboration.Results:Eight clinical trials, involving 504 patients, met the inclusion.The improvement of forced vital capacity as the main index of lung function in the experimental group was significantly higher than that in the control group (n SMD=0.94, 95%n CI: 0.74-1.13, n P<0.001). The change of forced expiratory volume in the first second as the secondary index was statistically significant (n SMD=1.06, 95%n CI: 0.83-1.29, n P<0.001). The incidence of adverse reactions was no significant difference between the experimental group and the control group (n OR=0.97, 95%n CI: 0.54-1.74, n P=0.91).n Conclusions:Pirfenidone combined with N-acetylcysteine is more effective than single-agent treatment in idiopathic pulmonary fibrosis, which delays the progression of the disease, has no increase on the incidence of adverse reactions.