Abstract

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.

Highlights

  • Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and the most common subtype of interstitial lung disease (ILD) characterized by dyspnea and progressive deterioration of lung function [1, 2]

  • Pulmonary rehabilitation (PR) had a significant effect on six-minute walk distance (6MWD) (MD:48.60; 95%confidence intervals (CI): 29.03 to 68.18; Z=4.87, P

  • The analysis showed a significant treatment effect on 6MWD (MD 48.60, 95% CI: 29.03 to 68.18, P

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Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and the most common subtype of interstitial lung disease (ILD) characterized by dyspnea and progressive deterioration of lung function [1, 2]. The incidence of IPF has been increasing worldwide, especially in Europe and North America and other developed countries. In these ones, the incidence was conservatively estimated to be 3 to 9 cases per 100,000 residents per year [3, 4]. Pharmacological therapy can relieve the symptoms and delay the decline of lung function of patients with IPF, the overall efficacy is still not satisfactory, and its cost and adverse effects cannot be ignored [10,11,12]. Pulmonary rehabilitation (PR) is one of the most popular treatments for nonpharmacological therapy, which has been recommended by guidelines for the treatment of patients with IPF; the evidence is still inconclusive [1, 13].

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