Pulmonary rehabilitation is recommended for patients with chronic lung diseases including idiopathic pulmonary fibrosis according to international guidelines. However, data for patients with interstitial lung disease (ILD) are limited. We examined the effect of an inpatient pulmonary rehabilitation on functional status and quality of life in ILD patients.We evaluated 402 consecutive ILD patients who were admitted to a specialised pulmonary rehabilitation centre (1999)(2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010). All patients performed a standardised pulmonary rehabilitation programme including pulmonary function tests, blood-gas analysis, 6-min walk test (6MWT), dyspnoea rating and health-related quality of life questionnaire (the 36-item short-form health survey; SF-36) on admission and discharge.Mean duration of pulmonary rehabilitation was 30¡1 days. 6MWT distance improved by 46¡3 m (308¡6 m versus 354¡6 m; p,0.001). Dyspnoea rating did not change. Lung function testing showed marginal improvement of vital capacity (+1¡0%; p50.002). The SF-36 questionnaire demonstrated an increase in all eight sub-scores as well as in the physical and mental health summary scores (physical 6¡1 points, p,0.001; mental health 10¡1 points, p,0.001). Moreover, patients with signs of pulmonary hypertension also benefited from pulmonary rehabilitation.In a large cohort of patients with ILD, pulmonary rehabilitation had a positive impact on functional status and quality of life. Considering the limited treatment options in this patient population pulmonary rehabilitation appears to be a valuable adjunct therapy. @ERSpublications Pulmonary rehabilitation improved functional status and QoL in a large cohort of ILD patients and appears to be a valuable adjunct therapy

Short-term comprehensive PR can significantly improve exercise capacity and HRQL in LTx candidates to a clinically relevant extent independent of the underlying lung disease. No relevant predictor influencing PR outcome could be detected. Further research is needed to evaluate the relevance of PR before LTx and its impact on clinical outcome after transplantation.

Our study supports the positive effects of PR in COPD and ILD patients. In both groups, patients with the biggest limitations benefit most from PR. However, relevant gender differences or other predictors could not be found.

BackgroundPulmonary rehabilitation (PR) following lung transplantation (LTx) is considered part of the optimal treatment in chronic obstructive pulmonary disease (COPD) for favourable post-operative outcomes. We investigated the effects of a PR intervention in the post-transplant phase with regard to lung function, exercise responses and cognitive function in COPD LTx recipients.Methods24 COPD LTx recipients (mean±sd forced expiratory volume in 1 s 75±22% predicted) were assigned to a comprehensive 3-week inpatient PR programme. Changes from PR admission to discharge in lung function variables, 6-min walk test-derived outcomes and cognitive function were assessed and examined for several factors. The magnitude of changes was interpreted by effect size (ES).ResultsIn response to the PR intervention, LTx recipients had improved lung function with regard to diffusing capacity of the lung for carbon monoxide (+4.3%; p=0.012) and static hyperinflation (residual volume/total lung capacity −2.3%; p=0.017), increased exercise capacity (6-min walk test +86 m; p<0.001), and had small to large improvements (ES range 0.23–1.00; all p≤0.34) in 50% of the administered cognitive tests. Learning skills and memory ability presented the greatest benefits (ES composite scores 0.62 and 0.31, respectively), which remained similar after stratification by single or bilateral LTx and sex.ConclusionsPR is an effective treatment for LTx recipients in the post-transplant phase, improving lung function, exercise responses, and domains of cognitive function of learning, memory and psychomotor speed. PR may facilitate the course of post-operative treatment and should be recommended in LTx.

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Lymphangioleiomyomatosis (LAM) is a rare and progressive cystic lung disease with limited therapeutic options. We retrospectively analyzed the effects of a comprehensive 4-week inpatient pulmonary rehabilitation (PR) program in 58 patients with advanced LAM (FEV1: 45 ± 34%predicted, 6-min walk distance (6MWD): 338 ± 167 m). Exercise performance (6MWD: + 49 ± 50 m; p < 0.001) and quality of life (SF-36 physical component: + 2.4 ± 7.8 points; p = 0.049 and mental component: + 5.2 ± 12.1 points; p < 0.001) increased significantly after PR comparable to an COPD cohort. There were no clinical parameters that predicted changes in outcomes following PR. PR seems to be an effective therapeutic option even in patients with advanced LAM. Trial registration Clinical-Trials registration number: NCT04184193; date of registration: December 3, 2019.

IntroductionAcute exacerbations of chronic obstructive pulmonary disease (AECOPD) are the most critical events for patients with COPD that have a negative impact on patients’ quality of life, accelerate disease progression, and can result in hospital admissions and death. Although there is no distinct definition or detailed knowledge about AECOPD, it is commonly used as primary outcome in clinical studies. Furthermore, it may be difficult in clinical practice to differentiate the worsening of symptoms due to an AECOPD or to the development of heart failure. Therefore, it is of major clinical importance to investigate the underlying pathophysiology, and if possible, predictors of an AECOPD and thus to identify patients who are at high risk for developing an acute exacerbation.Methods and analysisIn total, 355 patients with COPD will be included prospectively to this study during a 3-week inpatient pulmonary rehabilitation programme at the Schoen Klinik Berchtesgadener Land, Schoenau am Koenigssee (Germany). All patients will be closely monitored from admission to discharge. Lung function, exercise tests, clinical parameters, quality of life, physical activity and symptoms will be recorded, and blood samples and exhaled air will be collected. If a patient develops an AECOPD, there will be additional comprehensive diagnostic assessments to differentiate between cardiac, pulmonary or cardiopulmonary causes of worsening. Follow-up measures will be performed at 6, 12 and 24 months.Exploratory data analyses methods will be used for the primary research question (screening and identification of possible factors to predict an AECOPD). Regression analyses and a generalised linear model with a binomial outcome (AECOPD) will be applied to test if predictors are significant.Ethics and disseminationThis study has been approved by the Ethical Committee of the Philipps University Marburg, Germany (No. 61/19). The results will be presented in conferences and published in a peer-reviewed journal.Trial registration numberNCT04140097.

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