OBJECTIVE: To evaluate the efficacy and safety of pulmonary rehabilitation for exercise tolerance and quality of life improvement in idiopathic pulmonary fibrosis. METHODS: We searched PubMed, Cochrane Library, Embase, Web of Science, Chinese Biomedical Literature Database, China National Knowledge Infrastructure, Wanfang Database, and Chongqing VIP for randomized controlled trials that compared pulmonary rehabilitation with usual care for idiopathic pulmonary fibrosis. The risk of bias and certainty of evidence were assessed using Cochrane Collaboration's Risk of Bias tool and the Grading of Recommendations, Assessment, Development and Evaluation criteria. RESULTS: Eleven trials in total with 549 participants. Compared with usual care, pulmonary rehabilitation significantly increased 6-minute walking distance (mean difference 35.2m, 95% confidence intervals 25.4m to 44.9m; ten trials; 447 participants; moderate), decreased the St George's Respiratory Questionnaire total scores (mean difference -9.11, 95% confidence intervals -10.78 to -7.43; six trials; 303 participants; moderate), and reduced the modified Medical Research Council scores were lower (mean difference -0.76, 95% confidence intervals -1.25 to -0.27; three trials; 196 participants; low). Improvements were noted in forced vital capacity percent-predicted (mean difference 4.88, 95% confidence intervals 2.67 to 7.10; four trials; 214 participants; moderate) and diffusing capacity for carbon monoxide (mean difference 4.71, 95% confidence intervals 0.96 to 8.46; six trials; 358 participants; low). CONCLUSIONS: Pulmonary rehabilitation may significantly improve exercise tolerance and quality of life in idiopathic pulmonary fibrosis patients, but the quality of evidence was low to moderate. Large sample, multicenter, randomized controlled trials are needed to verify the efficacy and safety of pulmonary rehabilitation.

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