Background Idiopathic pulmonary fibrosis (IPF) is characterised by worsening dyspnoea and exercise intolerance. Research question Does a long-term pulmonary rehabilitation improve exercise tolerance in patients with IPF treated with standard antifibrotic drugs, which are expected to reduce disease progression? Methods This open-label randomised controlled trial was performed at 19 institutions. Stable patients receiving nintedanib were randomised into pulmonary rehabilitation and control groups (1:1). The pulmonary rehabilitation group underwent initial rehabilitation which included twice-weekly sessions of monitored exercise training for 12 weeks, followed by an at-home rehabilitation programme for 40 weeks. The control group received usual care only, without pulmonary rehabilitation. Both groups continued to receive nintedanib. The primary and main secondary outcomes were change in 6 min walking distance (6MWD) and change in endurance time (using cycle ergometry) at week 52. Results Eighty-eight patients were randomised into pulmonary rehabilitation (n=45) and control (n=43) groups. Changes in 6MWD were −33 m (95% CI −65 to −1) and −53 m (95% CI −86 to −21) in the pulmonary rehabilitation and control groups, respectively, with no statistically significant difference (mean difference, 21 m (95% CI −25 to 66), p=0.38). Changes in endurance time were significantly better in the pulmonary rehabilitation (64 s, 95% CI −42.3 to 171)) than in the control (−123 s (95% CI −232 to −13)) group (mean difference, 187 s (95% CI 34 to 153), p=0.019). Interpretation Although pulmonary rehabilitation in patients taking nintedanib did not improve 6MWD in the long term, it led to prolonged improvement in endurance time. Trial registration number UMIN000026376. Data are available on reasonable request.

中文翻译：

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肺康复对特发性肺纤维化的长期影响：一项随机对照试验

背景 特发性肺纤维化（IPF）的特点是呼吸困难恶化和运动不耐受。研究问题 长期肺康复是否可以改善接受标准抗纤维化药物治疗的 IPF 患者的运动耐量，这些药物有望减少疾病进展？方法 这项开放标签随机对照试验在 19 个机构进行。接受尼达尼布治疗的病情稳定的患者被随机分为肺康复组和对照组（1:1）。肺康复组接受了初步康复，其中包括每周两次的监测运动训练，持续 12 周，然后是家庭康复计划，持续 40 周。对照组仅接受常规护理，不进行肺康复治疗。两组均继续接受尼达尼布治疗。主要和主要次要结局是第 52 周时 6 分钟步行距离 (6MWD) 的变化和耐力时间的变化（使用自行车测功法）。 结果 88 名患者被随机分为肺康复组 (n=45) 和对照组 (n= 43）团体。肺康复组和对照组的6MWD变化分别为-33 m（95% CI -65至-1）和-53 m（95% CI -86至-21），无统计学显着差异（平均差， 21 m（95% CI -25 至 66），p=0.38）。肺康复组的耐力时间变化（64 秒，95% CI -42.3 至 -171））明显优于对照组（-123 秒（95% CI -232 至 -13））（平均差，187 秒） （95% CI 34 至 153），p=0.019）。解读 虽然服用尼达尼布的患者的肺康复从长远来看并没有改善 6MWD，它导致耐力时间的延长。试用注册号 UMIN000026376。可根据合理要求提供数据。