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目的:探讨N-乙酰半胱氨酸(N-acety-L-cysteine，NAC)对特发性肺纤维化(idiopathic pulmonary fibrosis，IPF)患者血清结缔组织生长因子(connective tissue growth factor，CTGF)和纤维连接蛋白(fibronectin，FN)水平的影响。方法:收集2016年1月至2018年12月，在昆山市第一人民医院收治且确诊为IPF患者150例，随机分为对照组和研究组，每组各75例。所有患者均接受常规治疗，研究组在上述基础上口服NAC。两组连续治疗6个月。记录并比较两组患者治疗前后的布罗格(Brog)呼吸困难评分、肺通气功能[肺活量占预计值百分比(prediced vital capacity，VC%pred)、用力肺活量占预计值百分比(prediced forced vital capacity，FVC%pred)]、血氧分压值(partial pressure of oxygen，PaOn 2)、一氧化碳弥散量值(diffusion capacity for carbon monoxide of the lung，DLco)，血清CTGF和FN的表达水平及NAC的治疗效果。n 结果:治疗前，对照组和研究组的Brog呼吸困难评分、VC%pred、FVC%pred、PaOn 2、Dlco、血清CTGF和FN表达水平差异无统计学意义(n P>0.05)。治疗后，对照组和研究组的Brog呼吸困难评分较治疗前明显降低，差异具有统计学意义(n t值分别为3.192和5.385，n P值均<0.05)；对照组和研究组的VC%pred和FVC%pred较治疗前明显增高，差异具有统计学意义(n t值分别为1.732和2.041，n P值均<0.05)；对照组和研究组CTGF和FN表达水平较治疗前明显降低，差异具有统计学意义(n t值分别为1.732，2.041，1.070和12.652，n P值均<0.05)；对照组和研究组的PaOn 2和DLco较治疗前明显增高，差异具有统计学意义(n t值分别为4.373，8.297，2.864和5.062，n P值均<0.05)。与对照组相比，治疗后观察组的Brog呼吸困难评分、CTGF和FN明显降低(n t值分别为2.128、5.196和45.445，n P值均<0.05)，VC%pred、FVC%pred、PaOn 2和DLco明显增高(n t值分别为3.241、3.625、3.337和2.280，n P值均<0.05)，差异均具有统计学意义。与对照组比，观察组的总有效率较高(n χ2＝4.160，n P0.05). While after treatment, Brog dyspnea scores in the control group and the study group were significantly lower than those before treatment (n t values were 3.192 and 5.385, respectively, all n P values <0.05); VC% PRED and FVC% PRED in the two groups were significantly higher than those before treatment ( n t values were 1.732 and 2.041, respectively, all n P values <0.05); CTGF and FN expression levels were significantly lower than those before treatment ( n t values were 1.732, 2.041, 1.070 and 12.652, respectively, all n P values <0.05); PaO n 2 and DLco were significantly higher than those before treatment (n t values were 4.373, 8.297, 2.864 and 5.062, respectively, all n P values <0.05). Compared with the control group, the Brog dyspnea score, expression of CTGF and FN were significantly decreased( n t values were 2.128, 5.196 and 45.445, respectively, all n P values <0.05), VC%pred, FVC%pred, PaO n 2 and DLco were significantly increased (n t values were 3.241, 3.625, 3.337 and 2.280, respectively, all n P values <0.05) in the study group after treatment.Compared with the control group, the total effective rate of the observation group was increased significantly( n χ2=4.160, n P<0.05). No significant adverse reactions occurred in either group.n Conclusion:The CTGF and FN in peripheral blood has significantly increased in IPF patients.NAC can significantly reduce the expression of CTGF and FN in peripheral blood, which suggested that NAC may have the effect of reducing the expression of CTGF and FN, but the mechanism still needs further study.