％0期刊文章％a ber，jürgen％a prasse，antje％a wirtz，hubert％a koschel，dirk％a pittrow，david％held，matthias％a klotsche，jens％a andreas，stefan a andreas，stefan％a claussen a claussen a claussen a martin％％A Grohé, Christian %A Wilkens, Henrike %A Hagmeyer, Lars %A Skowasch, Dirk %A Meyer, Joachim F. %A Kirschner, Joachim %A Gläser, Sven %A Kahn, Nicolas %A Welte, Tobias %A Neurohr,claus％schwaiblmair，Martin％A Bahmer，Thomas％A OQueka，Tim％A frankenberger，Marion％A KREUTER，MICHAEL％T存活和在存在或不存在抗纤维化治疗的患者的肺部纤维化治疗的情况下，长期肺纤维化：长- Insights-IPF注册表％D 2020％R 10.1183/13993003.02279-2019％J欧洲呼吸期刊％P 1902279％v 56％v 56％n 2％2％X目标缺乏抗纤维化疗法的抗纤维化疗法，（IPF）。我们旨在评估在现实生活中有和不接受抗纤维化疗法的IPF患者的疾病进程。方法分析了来自德国20个间隙肺部疾病专家中心的20个非惯用，前瞻性同类研究的数据。通过自动合理性检查，现场监控和源数据验证来确保数据质量。倾向得分被应用，以说明患有和不抗抗纤维化治疗的患者之间基线特征的已知差异。在适合分析的588例患者中，平均值±SD年龄为69.8±9.1岁，男性为81.0％。诊断以来的平均±SD疾病持续时间为1.8±3.4岁。强制生命力（FVC）和扩散能力（DLCO）的基线时的平均值±SD值分别为68.6±18.8％，预测分别为37.8±18.5％。在平均1.2±0.7岁的平均±SD随访中，194（33.0％）的患者死亡。对于患有抗纤维化疗法的患者而言，一年和2年的生存率分别为87％和46％和62％和62％和21％。 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 %U //www.qdcxjkg.com/content/erj/56/2/1902279.full.pdf