OBJECTIVE: The aim of this study is to evaluate the efficacy and safety of pulmonary rehabilitation (PR) in patients with idiopathic pulmonary fibrosis (IPF). METHODS: Embase, PubMed, Cochrane Library, China National Knowledge Infrastructure (CNKI), Chongqing VIP (CQVIP), Wanfang Data, and Chinese Biomedical Literature Database (SinoMed) were comprehensively searched. Randomized controlled trials (RCTs) that investigated the effects of PR for IPF patients were included. Literature selection and data extraction were conducted by two review authors independently. The Cochrane Collaboration's risk of bias tool and RevMan software (version 5.3) were used to evaluate the quality of studies and conduct statistical analysis, respectively. RESULTS: Seven studies (190 participants) were included. PR had a significant effect on six-minute walk distance (6MWD) (MD 48.60; 95%CI 29.03 to 68.18; z = 4.87, p < 0.00001), and 6MWD was improved more in subgroup analysis including studies conducted in Asia (MD 53.62; 95%CI 30.48 to 76.66; z = 4.54, p < 0.00001) and Europe (MD 54.10; 95% CI 26.65 to 101.56; z = 2.23, p = 0.03). Forced vital capacity (FVC%) was higher (MD 3.69; 95%CI 0.16 to 7.23; z = 2.05, p = 0.04). St George's Respiratory Questionnaire (SGRQ)/IPF-specific SGRQ (SGRQ-I) total score was lower (MD -7.87; 95% CI -11.44 to -4.30; z = 4.32, p < 0.0001). No significant effects were found for lung diffusing capacity determined by the single-breath technique (DLCO%) (MD 3.02; 95%CI -0.38 to 6.42; z = 1.74, p = 0.08). CONCLUSIONS: This study suggests that PR may enhance exercise capacity and improve quality of life in IPF patients. Besides, PR may also delay the decline of lung function of patients with IPF. However, further research should more fully assess the efficacy and safety of PR for IPF.

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