Trachalaki Athina, Lindahl Anna L, Petrarulo Simone, Margaritopoulos George A
Margaret Turner Warwick Centre for Fibrosing Lung Disease, National Heart and Lung Institute, Imperial College London, London, UK.
These authors contributed equally to this work.
Breathe (Sheff). 2025 Apr 17;21(2):240260. doi: 10.1183/20734735.0260-2024. eCollection 2025 Apr.
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with limited treatment options. Despite the approval of pirfenidone and nintedanib that slow disease progression, IPF remains a disease with poor survival. Promising therapeutic candidates were tested as potential treatments for IPF and while some drugs were successful in phase II clinical trials, their successful transition to positive phase III was unfortunately disappointing. This highlights the "regression to the truth" concept in drug development, whereby positive phase II trial results may simply be a statistical anomaly rather than the result of true efficacy. We examine three pivotal trials of novel IPF therapies, zinpentraxin alfa, ziritaxestat and pamrevlumab, that failed in late-stage clinical development. These failures underscore common pitfalls in IPF drug development, including inadequate phase II sample sizes, reliance on surrogate endpoints like forced vital capacity, and challenges integrating background antifibrotic therapies. Moving forward, innovative approaches like adaptive trial designs, Bayesian statistics and composite endpoints could improve trial robustness. Moreover, platform trials may accelerate drug development by testing multiple therapies simultaneously. Negative trials are not failures but opportunities for learning. By recognising and addressing these challenges, while also embracing novel trial methodologies, we can enhance drug development and improve IPF outcomes.
特发性肺纤维化(IPF)是一种治疗选择有限的慢性肺部疾病。尽管吡非尼酮和尼达尼布已获批可减缓疾病进展,但IPF仍然是一种生存率较低的疾病。有前景的治疗候选药物作为IPF的潜在治疗方法进行了测试,虽然一些药物在II期临床试验中取得了成功,但遗憾的是,它们向III期阳性结果的成功转化令人失望。这凸显了药物开发中的“回归真相”概念,即II期试验的阳性结果可能仅仅是一种统计异常,而非真正疗效的结果。我们研究了新型IPF疗法辛喷曲星α、齐瑞他司他和帕姆瑞单抗的三项关键试验,这些试验在后期临床开发中均告失败。这些失败凸显了IPF药物开发中常见的陷阱,包括II期样本量不足、依赖诸如用力肺活量等替代终点,以及整合背景抗纤维化疗法方面的挑战。展望未来,适应性试验设计、贝叶斯统计学和复合终点等创新方法可提高试验的稳健性。此外,平台试验可通过同时测试多种疗法来加速药物开发。阴性试验并非失败,而是学习的机会。通过认识并应对这些挑战,同时采用新型试验方法,我们可以加强药物开发并改善IPF的治疗效果。