Abstract
Purpose: Currently, pulmonary rehabilitation (PR) has a weak recommendation for idiopathic pulmonary fibrosis (IPF) and is often recommended for mild to moderate disease. We aimed to investigate the completion rate of PR in patients with advanced IPF, to analyze whether the severity of disease influences PR response and whether there is any difference between subjects who are able or not able to successfully complete the program.
Methods: Patients with IPF referred to lung transplantation (n = 48) were enrolled in an outpatient PR program including 3 times/wk supervised exercise training during 12 wk. A short-form 36-item health-related quality-of-life (HRQL) questionnaire and 6-min walk test distance (6MWD) were evaluated before and after PR.
Results: We found that 64.5% of patients successfully completed PR (31/48). Baseline forced vital capacity (FVC) and lung diffusion capacity for carbon monoxide (DLCO) were 49% +/- 13% and 46% +/- 17% of predicted, respectively. There was no difference comparing those who did and did not complete PR. In the former group, 6MWD (58 +/- 63 m) and several domains of the HRQL questionnaire improved significantly. No significant association was found between markers of disease severity (FVC, DLCO, and dyspnea) and improvement in clinical outcomes.
Conclusions: Two-thirds of our sample with advanced IPF referred to lung transplant successfully attended PR and improved exercise capacity and HRQL, without association with markers of disease severity. No difference was found at baseline compared with subjects who were not able to complete the program.