OBJECTIVE: To investigate the short and long-term effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis (IPF). DATA SOURCES: An electronic search of Medline, Embase and Cochrane Central databases along with hand search of relevant papers were performed on 15 March 2018. REVIEW METHODS: This study was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched the literature for randomized controlled trials of pulmonary rehabilitation in IPF patients. The outcomes were exercise capacity and health-related quality of life. Two authors independently extracted data, assessed trial eligibility and risk of bias. Meta-analyses were performed using RevMan and STATA software. RESULTS: We extracted data from four randomized controlled trials (142 participants). At short-term follow-up, pulmonary rehabilitation significantly enhanced 6-minute walk distance (6-MWD; weighted mean difference (WMD) 38.38, 95% confidence interval (CI) 4.64 to 72.12, I2 = 60.7%; p < 0.05), reduced St George's Respiratory Questionnaire (SGRQ)/IPF-specific SGRQ (SGRQ-I) total score (WMD -8.40, 95% CI -11.44 to -5.36, I2 = 0%; p < 0.00001). At long-term follow-up, pulmonary rehabilitation could not enhance 6-MWD (WMD 17.02, 95% CI -26.87 to 60.81, I2 = 36.3%; p = 0.43) or reduce SGRQ/SGRQ-I total score (WMD -3.45, 95% CI -8.55 to 1.64, I2 = 38.3%; p = 0.088). CONCLUSION: In patients with IPF, pulmonary rehabilitation showed short-term effects in enhancing exercise capacity and health-related quality of life, while it had no detectable effects at long-term follow-up.

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