Background: Idiopathic pulmonary fibrosis (IPF) is a chronic, devastating, interstitial lung disease, with few therapeutic options. IPF is characterized by pulmonary restriction, dyspnea, hypoxemia, exercise intolerance and poor quality of life (QOL). Objectives: We aimed to examine the effect of exercise training (ET) on clinical outcomes in IPF patients. Methods: A randomized controlled study included thirty-two IPF patients (aged 68 ± 8 years) who were allocated either to the ET group (n = 15), participating in a 12-week, twice-weekly 60-min supervised ET-based pulmonary rehabilitation program, or to a control group (n = 17) continuing with regular medical treatment alone. Cardiopulmonary exercise test, 6-min walking distance (6MWD) test, 30-second chair-stand test, pulmonary function tests, dyspnea and QOL were assessed at baseline and at the end of the 12-week intervention. Results: Significant differences were observed between the ET and the control groups in raw mean deltas (Δ = post- - pre-intervention): Δ6MWD, 81 m, p < 0.001; ΔVO₂ peak, 2.6 ml/kg/min, p = 0.002; Δwork rate, 22 W, p < 0.001; Δanaerobic threshold, 3.1 ml/kg/min, p < 0.001, and ΔFVC % predicted, 6%, p = 0.038. Dyspnea, QOL and 30-second chair-stand were also improved significantly following the program. Conclusions: ET improves exercise tolerance, functional capacity, pulmonary function, dyspnea and QOL in patients with IPF, suggesting a short-term treatment efficacy for clinical improvement, and should be considered the standard care for IPF.

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BACKGROUND: Lung transplantation (LTx) is an established treatment option for patients with end-stage lung diseases. Nevertheless, exercise intolerance, respiratory muscle function impairment, functional disability, and peripheral muscle weakness often persist following LTx. PURPOSE: To examine the effectiveness of a pulmonary rehabilitation (PR) program and home-based inspiratory muscle training (IMT) alone or in combination, in post lung transplantation (LTx) patients. METHODS: In a prospective pilot study a sample of 22 patients who had undergone LTx 4–18 months prior, were randomized to groups of PR alone, or combined with home-based IMT, or IMT alone, for six months: four months in the intervention program and two months of follow-up. Inspiratory muscle strength was assessed by measuring the maximal inspiratory pressure (MIP). RESULTS: There was a statistically significant increase in MIP values as well as in the 6-min-walk-test, only in the PR combined with home-based IMT, and IMT exercise groups. From baseline after 2- and 4-months intervention and at 6-months at follow-up, there was a statistically significant correlation between MIP and Maximum Voluntary Ventilation (MVV) values. CONCLUSIONS: Pulmonary rehabilitation (PR) program combined with inspiratory muscle training (IMT) or home-based IMT alone, in post lung transplantation patients, seems to be the best exercise combination for achieving the optimal effect, in inspiratory muscle strength and exercise capacity, with evidence for long-term benefits. Patients should be encouraged to participate in a PR program that includes home-based IMT exercise, especially in times of restricted mobility, as currently due to the Covid-19 pandemic.

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