The approvals of nintedanib and pirfenidone changed the treatment paradigm in idiopathic pulmonary fibrosis (IPF), and increased our understanding of the underlying disease mechanisms. Nonetheless, many challenges and unmet needs remain in the management of patients with IPF and other progressive fibrosing interstitial lung diseases.This review describes how the nintedanib clinical programme has helped to address some of these challenges. Data from this programme have informed changes to the IPF diagnostic guidelines, the timing of treatment initiation, and the assessment of disease progression. The use of nintedanib to treat patients with advanced lung function impairment, concomitant emphysema, patients awaiting lung transplantation and patients with IPF and lung cancer is discussed. The long-term use of nintedanib and an up-to-date summary of nintedanib in clinical practice are discussed. Directions for future research, namely emerging therapeutic options, precision medicine and other progressive fibrosing interstitial lung diseases, are described.Further developments in these areas should continue to improve patient outcomes.

中文翻译：

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肺纤维化的持续挑战和尼达尼布临床计划的见解。


尼达尼布和吡非尼酮的批准改变了特发性肺纤维化（IPF）的治疗模式，并增加了我们对潜在疾病机制的理解。尽管如此，在 IPF 和其他进行性纤维化间质性肺疾病患者的治疗中仍然存在许多挑战和未满足的需求。这篇综述介绍了尼达尼布临床计划如何帮助解决其中一些挑战。该计划的数据为 IPF 诊断指南、治疗开始时间和疾病进展评估的变化提供了信息。讨论了使用尼达尼布治疗晚期肺功能损伤、并发肺气肿的患者、等待肺移植的患者以及 IPF 和肺癌患者。讨论了尼达尼布的长期使用以及尼达尼布在临床实践中的最新总结。描述了未来研究的方向，即新兴的治疗选择、精准医学和其他进行性纤维化间质性肺疾病。这些领域的进一步发展应继续改善患者的治疗结果。