Background

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a fatal event that can occur during the clinical course of idiopathic pulmonary fibrosis (IPF). Although data from clinical trials suggest that the antifibrotic agents pirfenidone and nintedanib might reduce the risk of AE-IPF, the incidence of AE-IPF in patients receiving antifibrotic agents in clinical settings is unclear.

Objectives

To determine the incidence of AE-IPF in patients receiving antifibrotic agents and compare AE-IPF frequency in patients receiving pirfenidone and nintedanib.

Methods

We retrospectively reviewed the clinical records of 199 patients with IPF who were started on pirfenidone or nintedanib at our institution during the period from 2009 through 2018. Baseline characteristics, incidence of AE-IPF, and outcome after AE-IPF onset were analyzed.

Results

During the observation period, the 1-, 2-, and 3-year cumulative incidences of AE-IPF were 9.3 %, 22.1 %, and 25.0 %, respectively. The 1-, 2-, and 3-year cumulative incidence rates for AE-IPF in the pirfenidone group and nintedanib group were 5.1 % vs. 18.6 %, 20.4 % vs. 25.2 %, and 22.6 % vs. 29.6 %, respectively. AE-IPF incidence was significantly lower in patients treated with pirfenidone than in those treated with nintedanib (log rank test, P = 0.035). The 3-month survival rate after AE-IPF onset was 61.1 % in the pirfenidone group and 61.5 % in the nintedanib group; thus, outcomes after AE-IPF onset were similar in the 2 groups.

Conclusion

The reduction in AE-IPF risk might be greater for pirfenidone than for nintedanib.

中文翻译：

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接受抗纤维化药物治疗的患者特发性肺纤维化急性加重的发生率：真实世界经验

背景

特发性肺纤维化 (AE-IPF) 急性加重是特发性肺纤维化 (IPF) 临床过程中可能发生的致命事件。尽管来自临床试验的数据表明抗纤维化药物吡非尼酮和尼达尼布可能会降低 AE-IPF 的风险，但在临床环境中接受抗纤维化药物治疗的患者中 AE-IPF 的发生率尚不清楚。

目标

确定接受抗纤维化药物的患者的 AE-IPF 发生率，并比较接受吡非尼酮和尼达尼布的患者的 AE-IPF 频率。

方法

我们回顾性回顾了 2009 年至 2018 年期间在我们机构开始使用吡非尼酮或尼达尼布的 199 例 IPF 患者的临床记录。分析了基线特征、AE-IPF 的发生率和 AE-IPF 发作后的结果。

结果

在观察期间，AE-IPF 的 1 年、2 年和 3 年累积发生率分别为 9.3%、22.1% 和 25.0%。吡非尼酮组和尼达尼布组 AE-IPF 的 1 年、2 年和 3 年累积发病率分别为 5.1 % 对 18.6 %、20.4 % 对 25.2 % 和 22.6 % 对 29.6 %。接受吡非尼酮治疗的患者的 AE-IPF 发生率显着低于接受尼达尼布治疗的患者（对数秩检验，P = 0.035）。AE-IPF 发作后的 3 个月生存率在吡非尼酮组为 61.1%，在尼达尼布组为 61.5%；因此，AE-IPF 发病后的结果在 2 组中相似。

结论

吡非尼酮对 AE-IPF 风险的降低可能大于尼达尼布。