Interstitial Lung Diseases Center of Excellence, Department of Pulmonology, St Antonius Hospital, Koekoekslaan 1, Nieuwegein, 3435CM, The Netherlands.
Division of Heart and Lungs, University Medical Center Utrecht, Utrecht, The Netherlands.
Lung. 2023 Aug;201(4):335-343. doi: 10.1007/s00408-023-00628-4. Epub 2023 Jun 21.
Pirfenidone and nintedanib unequivocally inhibit FVC decline, but have been inconsistently linked to reduced mortality in phase III studies. On the contrary, real-world data show a survival benefit of antifibrotic drugs. However, it is unknown what this benefit is across different Gender, Age, and Physiology (GAP) stages.
Is there a difference in transplant-free (TPF) survival of IPF patients receiving antifibrotic drugs (IPF) compared with an untreated cohort (IPF)? Is this different for patients with GAP stage I, II, or III.
This is a single-center observational cohort study using prospectively included patients diagnosed with IPF between 2008-2018. Primary outcomes were TPF survival difference and 1-, 2-, and 3-year cumulative mortality for IPF and IPF. This was repeated after stratification for GAP stage.
In total, 457 patients were included. The median transplant-free survival was 3.4 years in IPF (n = 313) and 2.2 years in IPF (n = 144, p = 0.005). For GAP stage II, a median survival of 3.1 and 1.7 years was noted for IPF (n = 143) and IPF (n = 59, p < 0.001), respectively. A significantly lower 1-, 2-, and 3- year cumulative mortality was found for IPF with GAP stage II (1 yr: 7.0% vs 35.6%, 2 yr: 26.6% vs 55.9%, and 3 yr: 46.9% vs 69.5%). The 1-year cumulative mortality of IPF with GAP III was also significantly lower (19.0% vs 65.0%).
This large real-world study showed a survival benefit in IPF compared with IPF. This especially holds true for patients with GAP stage II and III.
吡非尼酮和尼达尼布明确抑制 FVC 下降，但在 III 期研究中与降低死亡率的关联不一致。相反，真实世界的数据显示抗纤维化药物具有生存获益。然而，尚不清楚在不同性别、年龄和生理（GAP）阶段的获益情况如何。
与未接受治疗的特发性肺纤维化（IPF）患者队列相比，接受抗纤维化药物（IPF）治疗的 IPF 患者在无移植（TPF）生存方面是否存在差异？在 GAP 分期为 I、II 或 III 的患者中是否存在差异？
这是一项单中心观察性队列研究，使用前瞻性纳入 2008-2018 年间诊断为 IPF 的患者。主要结局是 TPF 生存差异以及 IPF 和 IPF 的 1、2 和 3 年累积死亡率。对 GAP 分期进行分层后，重复该分析。
共纳入 457 例患者。IPF（n=313）和 IPF（n=144，p=0.005）的中位 TPF 生存时间分别为 3.4 年和 2.2 年。对于 GAP 分期 II，IPF（n=143）和 IPF（n=59）的中位生存时间分别为 3.1 年和 1.7 年，差异具有统计学意义（p<0.001）。对于 GAP 分期 II 的患者，IPF 的 1、2 和 3 年累积死亡率显著降低（1 年：7.0%比 35.6%，2 年：26.6%比 55.9%，3 年：46.9%比 69.5%）。GAP 分期 III 的 IPF 患者的 1 年累积死亡率也显著降低（19.0%比 65.0%）。
这项大型真实世界研究显示，与 IPF 相比，IPF 患者具有生存获益。对于 GAP 分期 II 和 III 的患者尤其如此。
