Short- and long-term effects of pulmonary rehabilitation for idiopathic pulmonary fibrosis: a systematic review and meta-analysis.

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) = 4.64-72.12, 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.