Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry
Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry
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DOI：
10.1183/13993003.02279-2019
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发表时间：
2020-08-01
影响因子：
24.3
通讯作者：
Kreuter, Michael
中科院分区：
文献类型：
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作者：
Behr, Juergen;Prasse, Antje;Kreuter, Michael
目的：特发性肺纤维化（IPF）抗纤维化治疗的观察数据缺乏。我们的目的是评估疾病的过程中的IPF患者和不抗纤维化治疗在现实生活conditions.Methods：我们分析了数据从一个非干预性，前瞻性队列研究的连续入组的IPF患者从20间质性肺病专家中心在德国。通过自动合理性检查、现场监测和源数据验证来确保数据质量。倾向分数被用来解释已知的基线特征的患者之间的差异和不抗纤维化therapy.Results：在588例适合分析，平均+/- so年龄为69.8 +/- 9.1岁，81.0%是男性。自诊断以来的平均疾病持续时间为1.8 +/- 3.4年。基线时用力肺活量（FVC）和弥散量（D-LCO）的平均+/- SD值分别为68.6 +/- 18.8%预测值和37.8 +/- 18.5%预测值。在平均± 0随访1.2 ± 0.7年期间，194例（33.0%）患者死亡。1年和2年生存率分别为87%与46%和62%与21%，与患者相比，抗纤维化治疗。抗纤维化治疗患者的死亡风险降低37%（风险比0.63，95% CI 0.45; 0.87; p=0.005）。在多变量分析中，结果是稳健的（并且保持统计学显著性）。FVC和D-LCO的总体下降是缓慢的，并没有显着差异患者或不抗纤维化therapeutic.Conclusions：生存率显着较高的IPF患者抗纤维化治疗，但肺功能参数的过程是相似的患者和不抗纤维化治疗。这表明，在临床实践中，尽管FVC和D-LCO测量值稳定，但IPF患者最终会发生过早死亡。
Objective: There is a paucity of observational data on antifibrotic therapy for idiopathic pulmonary fibrosis (IPF). We aimed to assess the course of disease of IPF patients with and without antifibrotic therapy under real-life conditions.Methods: We analysed data from a non-interventional, prospective cohort study of consecutively enrolled IPF patients from 20 interstitial lung disease expert centres in Germany. Data quality was ensured by automated plausibility checks, on-site monitoring, and source data verification. Propensity scores were applied to account for known differences in baseline characteristics between patients with and without antifibrotic therapy.Results: Among the 588 patients suitable for analysis, the mean +/- so age was 69.8 +/- 9.1 years, and 81.0% were male. The mean +/- so duration of disease since diagnosis was 1.8 +/- 3.4 years. The mean +/- SD value at baseline for forced vital capacity (FVC) and diffusion capacity (D-LCO) were 68.6 +/- 18.8% predicted and 37.8 +/- 18.5% predicted, respectively. During a mean +/- so 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 D-LCO 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 su vests that in clinical practice, premature mortality of IPF patients eventually occurs despite stable measurements for FVC and D-LCO.