TY - JOUR T1 -特发性肺纤维化患者在抗纤维化治疗存在或不存在时肺功能的生存和病程:长期结果INSIGHTS-IPF注册JF -欧洲呼吸杂志》乔和J - 10.1183/13993003.02279 -2019欧元六世- 56 - 2 SP - 1902279 AU Behr Jurgen盟——Prasse Antje盟,Wirtz休伯特盟——Koschel Dirk盟——Pittrow大卫非盟举行,马提亚盟——Klotsche Jens盟——安德烈亚斯,Stefan盟——Claussen马丁盟,高仪基督教盟,主教练威尔肯斯Henrike盟——Hagmeyer Lars AU - Skowasch Dirk AU -迈耶,约阿希姆f . AU -柯式,约阿希姆AU -格拉泽,斯文AU - Kahn,尼古拉斯盟——Welte Tobias盟——Neurohr老人盟——Schwaiblmair马丁AU - Bahmer,托马斯•AU - Oqueka蒂姆盟——私生子,马里昂AU - Kreuter如此说道,特发性肺纤维化(IPF)的抗纤维化治疗缺乏观察数据。我们的目的是评估现实生活条件下IPF患者接受和不接受抗纤维化治疗的病程。方法:我们分析了来自德国20个间质性肺病专家中心的连续入组IPF患者的非介入前瞻性队列研究数据。数据质量通过自动合理性检查、现场监控和源数据验证来保证。应用倾向评分来解释接受和未接受抗纤维化治疗的患者之间基线特征的已知差异。结果588例患者中，平均±sd年龄为69.8±9.1岁，男性占81.0%。自诊断以来的平均±sd病程为1.8±3.4年。基线时强迫肺活量(FVC)和扩散量(DLCO)的均值±sd分别为68.6±18.8%和37.8±18.5%。 During a mean±sd follow-up of 1.2±0.7 years, 194 (33.0%) patients died. The 1-year and 2-year survival rates were 87% versus 46% and 62% versus 21%, respectively, for patients with versus without antifibrotic therapy. The risk of death was 37% lower in patients with antifibrotic therapy (hazard ratio 0.63, 95% CI 0.45; 0.87; p=0.005). The results were robust (and remained statistically significant) on multivariable analysis. Overall decline of FVC and DLCO was slow and did not differ significantly between patients with or without antifibrotic therapy.Conclusions Survival was significantly higher in IPF patients with antifibrotic therapy, but the course of lung function parameters was similar in patients with and without antifibrotic therapy. This suggests that in clinical practice, premature mortality of IPF patients eventually occurs despite stable measurements for FVC and DLCO.Survival was significantly higher in antifibrotic-treated (AT) IPF patients, but the course of lung function parameters was similar in AT and non-AT patients, suggesting that functional stability alone may not safeguard against premature mortality in IPF https://bit.ly/2RDsrVY ER -