The Rationale, Evidence, and Adaptations to Pulmonary Rehabilitation for Chronic Respiratory Diseases Other Than COPD.

Over the last 3 decades, pulmonary rehabilitation (PR) has become an integral part of the management of COPD. Many other chronic respiratory diseases have similar systemic manifestations including skeletal muscle impairment, commonly through deconditioning, and may benefit from PR. However, whereas many programs may accept patients with other respiratory diseases, the program may need several adaptations to optimally manage patients. This article uses the examples of interstitial lung disease including idiopathic pulmonary fibrosis, bronchiectasis, pulmonary hypertension, post lung transplantation, and post-COVID condition to highlight exemplar clinical problems. In addition, the rationale and latest evidence for PR are described alongside the adaptations to the program, including education needs of the delivery team and close integrated care with the wider clinical team. Finally, future directions for clinical care and research are discussed.

Impacto de un programa de rehabilitación respiratoria domiciliario en la calidad de vida y ansiedad/depresión de pacientes con bronquiectasias no fibrosis quística / Impact of a home respiratory rehabilitation program on the quality of life and anxiety/depression of patients with bronchiectasia not cystic fibrosis

Introducción y objetivos: La valoración del efecto de la rehabilitación respiratoria domiciliaria (PRRD) en bronquiectasias no fibrosis quística (BQ no FQ) es un campo poco explorado hasta la fecha. El objetivo fue evaluar cómo influye un PRRD piloto en la disnea, la calidad de vida y en los trastornos del estado de ánimo así como su relación con la gravedad de la enfermedad. Material y métodos: Ensayo clínico no farmacológico en pacientes con BQ no FQ del Hospital Universitario Virgen Macarena. Se aleatorizó en: 1) grupo estudio (GE): programa de entrenamiento (resistencia y fuerza) en domicilio durante 8 semanas, 2) grupo control (GC): medidas educacionales por escrito. Se evaluó la gravedad de la enfermedad con el E-FACED, síntomas (cuestionario de Leicester (LCQ) y disnea (escala mMRC)), la calidad de vida (cuestionario de enfermedades respiratorias de Saint George (SGRQ)) y ansiedad y depresión (cuestionario hospitalario de ansiedad y depresión (HADS). Resultados: Después de 8 semanas en el GE existió mejoría en disnea de 0,46 ± 0,80, p = 0,010 y en la esfera física del LCQ de -0,68 ± 1,2, p = 0,043. Se produjo una mejoría en SGRQ actividad (-9 puntos, p = 0,025) y en el SGRQ total un cambio clínicamente relevante (-7 puntos, p = 0,063). La escala de depresión descendió 2,3 ± 4,2 puntos, p = 0,044. La gravedad no se relacionó con ninguna variable. Conclusiones: El PRRD mostró un claro beneficio en calidad de vida, síntomas y depresión de nuestros pacientes con BQ no FQ. (AU)

Introduction and objectives: the assessment of the effect of home-based pulmonary rehabilitation programmes (HPRP) in noncystic fibrosis bronchiectasis (non-CF BQ) is a field that has been little explored to date. Our objective was to evaluate how a pilot HPRP influences dyspnoea, quality of life and mood disorders and their relationship the severity of the disease. Material and methods: we present non-pharmacological clinical trial in patients with non-CF BQ at the Virgen Macarena University Hospital. It was randomized into1) study group (SG): received training program (resistance and strength) at home for 8 weeks and 2) control group (CG): received written educational measures. We assessed the impact of the program on disease severity (E-FACED), symptoms (Leicester Questionnaire (LCQ) and dyspnea (mMRC scale)), and quality of life (Saint George RespiratoryQuestionnaire) and anxiety and depression (Anxiety and Depression Hospital (HAD)). Results: after 8 weeks there was an improvement in dyspnoea of 0.46 ± 0,80, p = 0.010 and in the physical sphere of the LCQ of -0.68 ± 1.2, p = 0.043.There was an improvement in SGRQ activity (-9 points, p = 0.025) and in the total SGRQ a clinically relevant change (-7 points, p = 0.063).The depression scale decreased 2.3 ± 4.2 points, p = 0.044. There was no relationship between severity and any of the variables studied. Conclusions: the PRRD showed a clear benefit in quality of life, symptoms and depression of our patients with non-CF BQ. KeywordsMesH: Non-cystic fibrosis bronchiectasis, homebased respiratory rehabilitation, impact quality of life. (AU)

Respiratory Physiotherapy and Bronchiectasis / Fisioterapia respiratoria y bronquiectasias

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Effect of exercise-based pulmonary rehabilitation in patients with bronchiectasis: A meta-analysis.

OBJECTIVE: We aimed to assess the effectiveness of exercise training in patients with bronchiectasis in terms of exercise capacity, pulmonary function, and quality of life. METHODS: PubMed, Embase, and the Cochrane Library were searched for randomized controlled trials (RCTs) examining pulmonary rehabilitation to treat bronchiectasis, and the search timeline was from inception through November 2020. Two researchers independently screened the literature, extracted data, evaluated the risk of bias in the included studies, and used Review Manager 5.3 software to perform the meta-analysis. The primary outcomes were incremental shuttle walk distance (ISWD) and 6-minute walk distance (6-MWD) at 8 weeks. The secondary outcomes were forced expiratory volume in 1 second (FEV1), St. George's Respiratory Questionnaire (SGRQ) score, and Leicester Cough Questionnaire (LCQ) score at 8 weeks. RESULTS: A total of five RCTs with a total of 198 patients were included. A pooled analysis showed that improvements in ISWD (mean difference [MD] = 92.47 m, 95% confidence interval [CI] 49.87, 135.08; P < 0.0001), 6-MWD (MD = 31.01 m, 95% CI 1.60, 60.42; P = 0.04), and FEV1 (MD = 0.08 L, 95% CI 0.04, 0.12; P = 0.0002) in the pulmonary rehabilitation group were more marked than in the control group. There was no significant difference between the two groups in terms of the improvement in SGRQ and LCQ scores. CONCLUSIONS: Pulmonary rehabilitation improves exercise capacity and pulmonary function (specifically FEV1) in patients with bronchiectasis. However, due to limitations in the number and quality of current studies, the above conclusions need to be verified in future research.

Oral Positive Expiratory Pressure Device for Excessive Dynamic Airway Collapse Caused by Emphysema.

Excessive dynamic airway collapse (EDAC) contributes to breathlessness and reduced quality of life in individuals with emphysema. We tested a novel, portable, oral positive expiratory pressure (o-PEP) device in a patient with emphysema and EDAC. MRI revealed expiratory tracheal narrowing to 80 mm2 that increased to 170 mm2 with the o-PEP device. After 2-weeks use of the o-PEP device for 33% to 66% of activities, breathlessness, quality of life, and exertional dyspnea improved compared with minimal clinically important differences (MCID): University of California-San Diego Shortness of Breath questionnaire score declined 69 to 42 (MCID, ≥5), St. George's Respiratory Questionnaire score decreased 71 to 27 (MCID, ≥4), and before and after the 6-minute walk test Borg score difference improved from Δ3 to Δ2 (MCID, ≥1). During the 6-minute walk test on room air without the use of the o-PEP device, oxyhemoglobin saturation declined 91% to 83%; whereas, with the o-PEP device, the nadir was 90%. Use of the o-PEP device reduced expiratory central airway collapse and improved dyspnea, quality of life, and exertional desaturation in a patient with EDAC and emphysema.

Exercise training for bronchiectasis.

BACKGROUND: Bronchiectasis is characterised by excessive sputum production, chronic cough, and acute exacerbations and is associated with symptoms of dyspnoea and fatigue, which reduce exercise tolerance and impair quality of life. Exercise training in isolation or in conjunction with other interventions is beneficial for people with other respiratory diseases, but its effects in bronchiectasis have not been well established. OBJECTIVES: To determine effects of exercise training compared to usual care on exercise tolerance (primary outcome), quality of life (primary outcome), incidence of acute exacerbation and hospitalisation, respiratory and mental health symptoms, physical function, mortality, and adverse events in people with stable or acute exacerbation of bronchiectasis. SEARCH METHODS: We identified trials from the Cochrane Airways Specialised Register, ClinicalTrials.gov, and the World Health Organization trials portal, from their inception to October 2020. We reviewed respiratory conference abstracts and reference lists of all primary studies and review articles for additional references. SELECTION CRITERIA: We included randomised controlled trials in which exercise training of at least four weeks' duration (or eight sessions) was compared to usual care for people with stable bronchiectasis or experiencing an acute exacerbation. Co-interventions with exercise training including education, respiratory muscle training, and airway clearance therapy were permitted if also applied as part of usual care. DATA COLLECTION AND ANALYSIS: Two review authors independently screened and selected trials for inclusion, extracted outcome data, and assessed risk of bias. We contacted study authors for missing data. We calculated mean differences (MDs) using a random-effects model. We used the GRADE approach to assess the certainty of evidence. MAIN RESULTS: We included six studies, two of which were published as abstracts, with a total of 275 participants. Five studies were undertaken with people with clinically stable bronchiectasis, and one pilot study was undertaken post acute exacerbation. All studies included co-interventions such as instructions for airway clearance therapy and/or breathing strategies, provision of an educational booklet, and delivery of educational sessions. The duration of training ranged from six to eight weeks, with a mix of supervised and unsupervised sessions conducted in the outpatient or home setting. No studies of children were included in the review; however we identified two studies as currently ongoing. No data were available regarding physical activity levels or adverse events. For people with stable bronchiectasis, evidence suggests that exercise training compared to usual care improves functional exercise tolerance as measured by the incremental shuttle walk distance, with a mean difference (MD) between groups of 87 metres (95% confidence interval (CI) 43 to 132 metres; 4 studies, 161 participants; low-certainty evidence). Evidence also suggests that exercise training improves six-minute walk distance (6MWD) (MD between groups of 42 metres, 95% CI 22 to 62; 1 study, 76 participants; low-certainty evidence). The magnitude of these observed mean changes appears clinically relevant as they exceed minimal clinically important difference (MCID) thresholds for people with chronic lung disease. Evidence suggests that quality of life improves following exercise training according to St George's Respiratory Questionnaire (SGRQ) total score (MD -9.62 points, 95% CI -15.67 to -3.56 points; 3 studies, 160 participants; low-certainty evidence), which exceeds the MCID of 4 points for this outcome. A reduction in dyspnoea (MD 1.0 points, 95% CI 0.47 to 1.53; 1 study, 76 participants) and fatigue (MD 1.51 points, 95% CI 0.80 to 2.22 points; 1 study, 76 participants) was observed following exercise training according to these domains of the Chronic Respiratory Disease Questionnaire. However, there was no change in cough-related quality of life as measured by the Leicester Cough Questionnaire (LCQ) (MD -0.09 points, 95% CI -0.98 to 0.80 points; 2 studies, 103 participants; moderate-certainty evidence), nor in anxiety or depression. Two studies reported longer-term outcomes up to 12 months after intervention completion; however exercise training did not appear to improve exercise capacity or quality of life more than usual care. Exercise training reduced the number of acute exacerbations of bronchiectasis over 12 months in people with stable bronchiectasis (odds ratio 0.26, 95% CI 0.08 to 0.81; 1 study, 55 participants). After an acute exacerbation of bronchiectasis, data from a single study (N = 27) suggest that exercise training compared to usual care confers little to no effect on exercise capacity (MD 11 metres, 95% CI -27 to 49 metres; low-certainty evidence), SGRQ total score (MD 6.34 points, 95%CI -17.08 to 29.76 points), or LCQ score (MD -0.08 points, 95% CI -0.94 to 0.78 points; low-certainty evidence) and does not reduce the time to first exacerbation (hazard ratio 0.83, 95% CI 0.31 to 2.22). AUTHORS' CONCLUSIONS: This review provides low-certainty evidence suggesting improvement in functional exercise capacity and quality of life immediately following exercise training in people with stable bronchiectasis; however the effects of exercise training on cough-related quality of life and psychological symptoms appear to be minimal. Due to inadequate reporting of methods, small study numbers, and variation between study findings, evidence is of very low to moderate certainty. Limited evidence is available to show longer-term effects of exercise training on these outcomes.

Efficacy of pulmonary rehabilitation for bronchiectasis and related factors: which patients should receive the most treatment?

INTRODUCTION: Pulmonary rehabilitation (PR) is an effective approach for patients with chronic pulmonary disease, and it is also recommended for patients with bronchiectasis. The aims of the current study were to evaluate the efficacy of a multidisciplinary PR program and identify factors associated with improvement in patients with bronchiectasis. Material and ethods: We obtained data from patients with bronchiectasis who completed our PR program which consisted of education and training regarding bronchial hygiene. Pulmonary function test results, body composition, exercise capacity, quality of life, and psychological status were assessed before and after the PR program. RESULTS: We enrolled 130 patients in this retrospective study. Most patients had a history of pneumonia. The Medical Research Council (MRC) dyspnea scale, incremental shuttle walking test (ISWT), endurance shuttle walking test (ESWT), St. George's Respiratory Questionnaire (SGRQ), Chronic Respiratory Questionnaire (CRQ), and Hospital Anxiety and Depression (HAD) scores statistically improved after the PR program (all p < 0.001). Improvements were similar regardless of sex, etiology, smoking sta-tus, or number of hospitalizations. Age was negatively correlated with ΔSGRQ (p = 0.024, r = -0.203). Baseline forced expiratory volume in 1s (FEV1) was positively correlated with ΔCRQ (p = 0.015, r = 0.213) and negatively correlated with Δanxiety (p = 0.014, r = -0.215). Baseline MRC was negatively correlated with ΔMRC (p < 0.001, r = -0.563) and ΔSGRQ (p < 0.001, r = -0.308). Baseline ISWT was negatively correlated with ΔISWT (p = 0.043, r = -0.176) and Δanxiety (p = 0.007, r = -0.237). Baseline SGRQ was negatively correlated with ΔMRC (p = 0.003, r = -0.267) and ΔSGRQ (p < 0.001, r = -0.648). CONCLUSIONS: Our PR program is efficacious for patients with bronchiectasis regardless of sex, etiologic cause of bronchiectasis, concomitant chronic obstructive pulmonary disease, smoking status, and/or number of hospitalizations. Improvement varied among patients which highlights the need for more studies to determine which patients will benefit most from the program.

Application of the Modified Incremental Step Test for Pulmonary Rehabilitation.

OBJECTIVE: A Modified Incremental Step Test (MIST) performed in the home may facilitate entirely home-based pulmonary rehabilitation programs. The aims of this study were to investigate the reliability and responsiveness, and the utility of the MIST for exercise prescription in people with stable chronic lung disease. METHODS: The MIST was undertaken at the center and home in random order, before and after pulmonary rehabilitation, with 2 tests at each time point. Reliability was assessed using intraclass correlation coefficient. Responsiveness was evaluated as effect size. The minimal important difference was appraised using distribution and anchor-based methods. In a substudy, physiological responses to MIST were measured by a portable metabolic system, followed by a constant step rate test at 60% of peak oxygen uptake (VO2peak), to evaluate utility for exercise prescription. RESULTS: Forty-six participants were recruited (29% of eligible candidates). There was excellent reliability for number of steps recorded in home- and center-based settings (intraclass correlation coefficient = 0.954, 95% CI = 0.915-0.976). A small-moderate effect size was demonstrated following pulmonary rehabilitation (0.34), and the minimal detectable change was 7 steps. All participants in the substudy achieved 60% of VO2peak and achieved steady state by the fourth minute, with 60% of VO2peak corresponding to a mean 37% (95% CI = 29-44) of the MIST final level. CONCLUSIONS: The MIST is reliable and responsive to pulmonary rehabilitation in people with stable chronic respiratory disease. It provides new opportunities to assess exercise capacity, prescribe exercise training, and reassess exercise program outcomes in environments where established field walking tests are not feasible. IMPACT: Pulmonary rehabilitation is a highly effective treatment that is underutilized worldwide. Home-based pulmonary rehabilitation may improve access for patients and deliver equivalent clinical outcomes but is limited by the availability of a robust exercise test that can be used at home to assess exercise capacity and prescribe training intensity. This study tested the clinimetric properties of the MIST and demonstrated a new way to assess exercise capacity, prescribe exercise training of an appropriate intensity, and reassess exercise capacity in environments where established field walking tests are not feasible.

Anxiety and depression in bronchiectasis: Response to pulmonary rehabilitation and minimal clinically important difference of the Hospital Anxiety and Depression Scale.

The aims of the study were to evaluate the responsiveness of Hospital Anxiety and Depression Scale-Anxiety (HADS-A) subscale and HADS-Depression (HADS-D) subscale to pulmonary rehabilitation (PR) in patients with bronchiectasis compared to a matched group of patients with chronic obstructive pulmonary disease (COPD) and provide estimates of the minimal clinically important difference (MCID) of HADS-A and HADS-D in bronchiectasis. Patients with bronchiectasis and at least mild anxiety or depression (HADS-A ≥ 8 or/and HADS-D ≥ 8), as well as a propensity score-matched control group of patients with COPD, underwent an 8-week outpatient PR programme (two supervised sessions per week). Within- and between-group changes were calculated in response to PR. Anchor- and distribution-based methods were used to estimate the MCID. HADS-A and HADS-D improved in response to PR in both patients with bronchiectasis and those with COPD (median (25th, 75th centile)/mean (95% confidence interval) change: HADS-A change: bronchiectasis -2 (-5, 0), COPD -2 (-4, 0); p = 0.43 and HADS-D change: bronchiectasis -2 (-2 to -1), COPD -2 (-3 to -2); p = 0.16). Using 26 estimates, the MCID for HADS-A and HADS-D was -2 points. HADS-A and HADS-D are responsive to PR in patients with bronchiectasis and symptoms of mood disorder, with an MCID estimate of -2 points.

Oxidative and inflammatory effects of pulmonary rehabilitation in patients with bronchiectasis. A prospective, randomized study / Efectos oxidativos e inflamatorios de la rehabilitación pulmonar en pacientes con bronquiectasia. Estudio prospectivo aleatorizado

Background: systemic inflammation and oxidative stress are important factors in the pathogenesis of bronchiectasis. Pulmonary rehabilitation (PR) is recommended for bronchiectasis, but there is no data about its effect on the inflammatory and REDOX status of these patients. Aims: to investigate the effect of PR in non-cystic-fibrosis bronchiectasis (NCFB) patients, and to compare it with the effect of PR plus a hyperproteic oral nutritional supplement (PRS) enriched with beta-hydroxy-beta-methylbutyrate (HMB) on serum inflammatory and oxidative biomarkers. Materials and methods: this was an open randomized, controlled trial. Thirty individuals (65 years old or younger with a body mass index over 18.5, older than 65 years with a body mass index over 20) were recruited from September 2013 to September 2014, and randomly assigned to receive PR or PRS. Total neutrophils, and inflammatory and oxidative biomarker levels were measured at baseline, and then at 3 and 6 months. Results: in the PRS group neutrophil levels were decreased from baseline at 6 months. A significantly different fold change was found between the PR and PRS groups. In the PR group, IL-6 and adiponectin were increased by the end of the study while TNFa levels were decreased from baseline at 6 months. REDOX biomarkers remained stable throughout the study except for 8-isoprostane levels, which were increased from baseline at 6 months in both groups of patients. Conclusion: a PR program induced a pro-oxidative effect accompanied by changes in circulating inflammatory cytokine levels in NCFB patients. Our results would also suggest a possible beneficial effect of the HMB enriched supplement on neutrophil level regulation in these patients. The information provided in this study could be useful for choosing the right therapeutic approach in the management of bronchiectasis

Introducción: la inflamación sistémica y el estrés oxidativo son factores importantes en la patogénesis de la bronquiectasia. La rehabilitación pulmonar (PR) está recomendada en los sujetos con bronquiectasias, pero no hay datos sobre sus posibles efectos sobre el estado inflamatorio y REDOX de estos pacientes. Objetivos: investigar el efecto de la PR en pacientes con bronquiectasias no asociadas a fibrosis quística (NCFB) sobre los biomarcadores oxidativos e inflamatorios, y compararlo con los efectos de la PR junto con la suplementación oral de un suplemento hiperproteico (PRS) enriquecido con beta-hidroxi-beta-metilbutirato (HMB). Material y métodos: ensayo clínico abierto, aleatorizado y controlado. Treinta pacientes (de 65 años o menos con un índice de masa corporal por encima de 18,5, y mayores de 65 años con un índice de masa corporal de más de 20) se aleatorizaron para recibir PR o PRS. Los niveles circulantes de neutrófilos totales y los de biomarcadores de estado inflamatorio y oxidativo se determinaron al inicio del estudio y a los 3 y 6 meses. Resultados: los niveles de neutrófilos en el grupo de PRS se redujeron desde el inicio a los 6 meses, presentando una tasa de cambio significativamente diferente según el tratamiento. En el grupo de PR, la IL-6 y la adiponectina aumentaron al final del estudio, mientras que los niveles de TNFa disminuyeron desde el inicio a los 6 meses. Los biomarcadores de estrés oxidativo se mantuvieron estables durante todo el estudio excepto por los niveles de 8-isoprostano, que aumentaron desde el inicio a los 6 meses en ambos grupos de pacientes. Conclusión: el programa de PR indujo un efecto pro-oxidativo acompañado de cambios en los niveles de citoquinas inflamatorias circulantes en pacientes con NCFB. Nuestros resultados también sugieren un posible efecto beneficioso del suplemento nutricional sobre la regulación de los niveles de neutrófilos de estos pacientes

Oxidative and inflammatory effects of pulmonary rehabilitation in patients with bronchiectasis. A prospective, randomized study.

INTRODUCTION: Background: systemic inflammation and oxidative stress are important factors in the pathogenesis of bronchiectasis. Pulmonary rehabilitation (PR) is recommended for bronchiectasis, but there is no data about its effect on the inflammatory and REDOX status of these patients. Aims: to investigate the effect of PR in non-cystic-fibrosis bronchiectasis (NCFB) patients, and to compare it with the effect of PR plus a hyperproteic oral nutritional supplement (PRS) enriched with beta-hydroxy-beta-methylbutyrate (HMB) on serum inflammatory and oxidative biomarkers. Materials and methods: this was an open randomized, controlled trial. Thirty individuals (65 years old or younger with a body mass index over 18.5, older than 65 years with a body mass index over 20) were recruited from September 2013 to September 2014, and randomly assigned to receive PR or PRS. Total neutrophils, and inflammatory and oxidative biomarker levels were measured at baseline, and then at 3 and 6 months. Results: in the PRS group neutrophil levels were decreased from baseline at 6 months. A significantly different fold change was found between the PR and PRS groups. In the PR group, IL-6 and adiponectin were increased by the end of the study while TNFα levels were decreased from baseline at 6 months. REDOX biomarkers remained stable throughout the study except for 8-isoprostane levels, which were increased from baseline at 6 months in both groups of patients. Conclusions: a PR program induced a pro-oxidative effect accompanied by changes in circulating inflammatory cytokine levels in NCFB patients. Our results would also suggest a possible beneficial effect of the HMB enriched supplement on neutrophil level regulation in these patients. The information provided in this study could be useful for choosing the right therapeutic approach in the management of bronchiectasis.

INTRODUCCIÓN: Introducción: la inflamación sistémica y el estrés oxidativo son factores importantes en la patogénesis de la bronquiectasia. La rehabilitación pulmonar (PR) está recomendada en los sujetos con bronquiectasias, pero no hay datos sobre sus posibles efectos sobre el estado inflamatorio y REDOX de estos pacientes. Objetivos: investigar el efecto de la PR en pacientes con bronquiectasias no asociadas a fibrosis quística (NCFB) sobre los biomarcadores oxidativos e inflamatorios, y compararlo con los efectos de la PR junto con la suplementación oral de un suplemento hiperproteico (PRS) enriquecido con beta-hidroxi-beta-metilbutirato (HMB). Material y métodos: ensayo clínico abierto, aleatorizado y controlado. Treinta pacientes (de 65 años o menos con un índice de masa corporal por encima de 18,5, y mayores de 65 años con un índice de masa corporal de más de 20) se aleatorizaron para recibir PR o PRS. Los niveles circulantes de neutrófilos totales y los de biomarcadores de estado inflamatorio y oxidativo se determinaron al inicio del estudio y a los 3 y 6 meses. Resultados: los niveles de neutrófilos en el grupo de PRS se redujeron desde el inicio a los 6 meses, presentando una tasa de cambio significativamente diferente según el tratamiento. En el grupo de PR, la IL-6 y la adiponectina aumentaron al final del estudio, mientras que los niveles de TNFα disminuyeron desde el inicio a los 6 meses. Los biomarcadores de estrés oxidativo se mantuvieron estables durante todo el estudio excepto por los niveles de 8-isoprostano, que aumentaron desde el inicio a los 6 meses en ambos grupos de pacientes. Conclusión: el programa de PR indujo un efecto pro-oxidativo acompañado de cambios en los niveles de citoquinas inflamatorias circulantes en pacientes con NCFB. Nuestros resultados también sugieren un posible efecto beneficioso del suplemento nutricional sobre la regulación de los niveles de neutrófilos de estos pacientes.

Pulmonary rehabilitation adapted index of self-efficacy (PRAISE) validated to Portuguese respiratory patients.

INTRODUCTION AND OBJECTIVES: Recent updates on Pulmonary Rehabilitation highlight the importance of patients' self-efficacy on long-term adherence to health-enhancing behaviors. The Pulmonary Rehabilitation Adapted Index of Self-Efficacy (PRAISE) is an adaptation of the General Self-Efficacy Scale. This study aimed to translate, culturally adapt and evaluate reliability and validity of PRAISE in Portuguese respiratory patients. PATIENTS OR MATERIALS AND METHODS: Forward-backward translation and pilot testing were performed. Content validity was assessed by a multidisciplinary panel of expert judges. To evaluate reliability and validity, 150 respiratory outpatients on Pulmonary Rehabilitation participated in a cross-sectional study. Descriptive and reliability analyses, and exploratory factorial analysis using principal axis factoring, followed by oblique oblimin factor rotation were conducted to identify construct validity. IBM® SPSS® version 22 was used to perform statistical analysis. RESULTS: 150 patients with a mean age of 67 years, 54% male and 83% currently on Pulmonary Rehabilitation participated in the study. These included mainly Chronic Obstructive Pulmonary Disease patients (46.7%) but also Bronchiectasis (20%), Interstitial Lung Disease (20%) and other respiratory diseases. PRAISE mean score was 49. Exploratory factor analysis extraction provided a 4-factor solution that cumulatively explained 52.3% of total variance (F1: 26.6%; F2: 9.7%; F3: 8.7%; F4: 7.3%). Portuguese PRAISE showed a reliability of 0.78 (Chronbach alpha). CONCLUSIONS: The Portuguese version of PRAISE showed adequate psychometric properties for it to be used as an instrument to measure self-efficacy as a patient-centered outcome on Pulmonary Rehabilitation.

Pulmonary rehabilitation after exacerbation of bronchiectasis: a pilot randomized controlled trial.

BACKGROUND: Pulmonary rehabilitation improves exercise capacity and reduces risk of future exacerbation in COPD when performed after an exacerbation. There have been no previous studies of post-exacerbation rehabilitation in bronchiectasis. METHODS: Parallel group randomized controlled trial compared pulmonary rehabilitation (PR) to standard care (SC) in patients followed an antibiotic treated exacerbation of bronchiectasis. Patients were randomized following a 14 day course of antibiotics was completed. The primary outcome was 6-min walk distance (6 MW) at 8 weeks. Secondary outcomes were time to the next exacerbation, St.Georges Respiratory Questionnaire, COPD CAT score, Leicester cough questionnaire (LCQ) and FEV1 at 8 and 12 weeks post exacerbation. RESULTS: Forty eight patients were enrolled but only 27 had exacerbations within 12 months of enrolment. Nine patients received pulmonary rehabilitation and 18 received standard care. The 6 MW improved significantly from post-exacerbation to 8 weeks in both groups, with no significant difference between PR and SC- mean difference of 11 m (95% CI -34.3 to 56.3,p = 0.6). Time to the next exacerbation was not significantly different hazard ratio 0.83 (0.31-2.19, p = 0.7). No significant differences were seen between groups in terms of LCQ, CAT, FEV1 or SGRQ between groups. An analysis of probability based on the patients enrolled suggested > 1000 subjects are likely be required to have an > 80% probability of observing a statistically significant difference between PR and SC and any such differences would be likely to be too small to be clinically relevant. CONCLUSIONS: This pilot study identified no significant benefits associated with pulmonary rehabilitation after exacerbations of bronchiectasis. TRIAL REGISTRATION: NCT02179983, registered on Clinicaltrials.gov 29th June 2014.

Practical approach to establishing pulmonary rehabilitation for people with non-COPD diagnoses.

Pulmonary rehabilitation is a core aspect in the management of patients with chronic respiratory diseases. This paper describes a practical approach to establishing pulmonary rehabilitation for patients with non-COPD diagnoses using examples from the interstitial lung disease (ILD), pulmonary hypertension (PH), bronchiectasis and lung cancer patient populations. Aspects of pulmonary rehabilitation, including the rationale, patient selection, setting of programmes, patient assessment and training components (both exercise and non-exercise aspects), are discussed for these patient groups. Whilst there are many similarities in the rationale and application of pulmonary rehabilitation across these non-COPD populations, there are also many subtle differences, which are discussed in detail in this paper. With consideration of these factors, pulmonary rehabilitation programmes can be adapted to facilitate the inclusion of respiratory patients with non-COPD diagnoses.

National Survey on the Management of Adult Bronchiectasis in Belgium.

The increasing prevalence and incidence of bronchiectasis leads to a substantial health care burden. Quality standards for the management of bronchiectasis were formulated by the British Thoracic Society following publication of guidelines in 2010. They can be used as a benchmark for quality of care. It is, however, unclear how and whether they apply outside of the UK. Between May and November 2017, we conducted an online survey among respiratory physicians caring for adult bronchiectasis patients in Belgium. About 186 cases were submitted by 117 treating physicians. Patients were mostly female (58%), of Caucasian descent (84%) with a remarkably low median age of 59.8 (IQR 47-73) years. 41% had Pseudomonas aeruginosa, methicillin-resistant Staphylococcus aureus and/or Enterobacteriaceae isolated from respiratory samples in the past. 21% had three or more exacerbations, however, more than 58% were receiving long-term oral antibiotics (of which 90% azithromycin). In 40% of patients the diagnostic testing was insufficient. Surveillance of sputum bacteriology in stable patients and composing a self-management plan was missing in 53% and 68% of patients, respectively. Airway clearance techniques were implemented in 84%. Respiratory physicians complied with 60% or more to five out of the eight applicable quality standards, which is encouraging. Increasing educational act could further raise awareness and increase quality of care.

Airway clearance, mucoactive therapies and pulmonary rehabilitation in bronchiectasis.

This paper aims to provide physiological rationale for airway clearance, mucoactive therapy and pulmonary rehabilitation (PR) (or exercise interventions) in bronchiectasis. There is increasing emphasis on the role of airway clearance techniques (ACT) in the management of bronchiectasis. No single ACT has currently shown superior effect over another. Given the large range of different techniques available, consideration of the physiological effects underpinning a technique including expiratory flow, ventilation and oscillation, is essential to effectively personalize ACT. Key clinical trials of mucoactives in bronchiectasis are underway and will provide clarity on the role of these agents in the management of patients with bronchiectasis. Prescription of mucoactive therapies should be done in conjunction with ACT and therefore the mechanism of action of mucoactive drugs and their timing with ACT should be taken into consideration. PR and/or exercise training are recommended in all current bronchiectasis guidelines. There is a clear physiological rationale that muscle weakness and physical inactivity may play a role in disease progression as well as impacting health-related quality of life, frequency of pulmonary exacerbations and ability to mobilize sputum. However, there are residual unanswered questions surrounding the delivery and accessibility to PR. This review summarizes the physiological principles and supporting evidence for airway clearance, mucoactive medication and PR, which are key components in the management of bronchiectasis.

Objectively measured physical activity and sedentary behaviour in children with bronchiectasis: a cross-sectional study.

BACKGROUND: Bronchiectasis is a major contributor to respiratory morbidity and health care utilization in children and youth. Current treatment guidelines for bronchiectasis recommend participation in regular physical activity (PA) to improve aerobic fitness and quality of life (QoL). However, no previous study has assessed physical activity and sedentary behavior in this patient group, and the extent to which children with bronchiectasis meet guidelines for PA is unknown. In the absence of such data, we objectively measured the PA of children with bronchiectasis and compared them to current guidelines. METHODS: Forty-six children with bronchiectasis between 4 to 14 years (mean age 7.5 ± 2.6 years) were recruited from the Queensland Children's Hospital, Brisbane. Daily time in sedentary, light, and moderate-to-vigorous PA (MVPA) was measured objectively over 7 days using the ActiGraph GT3X+ accelerometer and compared their values to current guidelines (minimum 60 min of MVPA daily). Compliance with the daily guideline and average daily steps counts were compared to normative data from two population-based health surveys of healthy children. RESULTS: We had complete measurements from 36 children. On average, they accumulated 48.6 min of MVPA daily and were sedentary for ~ 7 h/day. There was no statistical difference in these values between sexes or weekdays vs. weekends. Only 2 (5.6%) children met the 60-min daily MVPA recommendation compared to 42.1% of healthy children. Children with bronchiectasis accumulated 8229 steps/day (boys: 8422 ± SD 473, girls: 8037 ± 594), well below the recommended 12,000 steps/day. In comparison, daily step counts in healthy children ranged from 11,500-14,500 steps/day. CONCLUSION: Children with bronchiectasis are insufficiently active for health benefit and would substantially benefit from programs to promote PA and reduce sedentary behavior.

High-intensity inspiratory muscle training in bronchiectasis: A randomized controlled trial.

BACKGROUND AND OBJECTIVE: Inspiratory muscle training (IMT) enhances velocity of inspiratory muscle contraction and modifies inspiratory and expiratory time. This study aimed to examine the impact of high-intensity IMT (H-IMT) on exercise capacity in bronchiectasis. METHODS: Forty-five patients were included. Lung function, respiratory muscle strength and endurance, exercise capacity, dyspnoea, fatigue and quality of life (QOL) were evaluated. Patients were randomized into two groups: H-IMT and control groups. Twenty-three patients underwent H-IMT for 8 weeks, using threshold loading with a target workload of maximal inspiratory pressure (MIP) of at least 70%, with 3-min cycles (as 2-min training: 1-min rest intervals) for 21 min. There was a total period of 14 min of loaded breathing and 7 min of recovery. The control group (n = 22) underwent low-intensity IMT at 10% of the initial MIP and was maintained at the same intensity until the end of the training. RESULTS: After training, both MIP and maximal expiratory pressure (MEP) and the incremental shuttle walk distance were increased in the H-IMT group compared with the control group (P < 0.05). There was a significant difference in constant threshold load, time and pressure-time units in the H-IMT group (P < 0.05) but not in the control group (P > 0.05). A significant decrease was found in fatigue in both groups (P < 0.05). The Leicester Cough Questionnaire social score for the H-IMT group decreased significantly after the treatment (P < 0.05). CONCLUSION: The H-IMT increased exercise capacity in patients with non-cystic fibrosis bronchiectasis. It has also positive effects on respiratory muscle strength and endurance, and social aspects of QOL.

Pulmonary rehabilitation in bronchiectasis: a propensity-matched study.

International guidelines recommend pulmonary rehabilitation for patients with bronchiectasis, supported by small trials and data extrapolated from chronic obstructive pulmonary disease (COPD). However, it is unknown whether real-life data on completion rates and response to pulmonary rehabilitation are similar between patients with bronchiectasis and COPD.Using propensity score matching, 213 consecutive patients with bronchiectasis referred for a supervised pulmonary rehabilitation programme were matched 1:1 with a control group of 213 patients with COPD. Completion rates, change in incremental shuttle walk (ISW) distance and change in Chronic Respiratory Disease Questionnaire (CRQ) score with pulmonary rehabilitation were compared between groups.Completion rate was the same in both groups (74%). Improvements in ISW distance and most domains of the CRQ with pulmonary rehabilitation were similar between the bronchiectasis and COPD groups (ISW distance: 70 versus 63 m; CRQ-Dyspnoea: 4.8 versus 5.3; CRQ-Emotional Function: 3.5 versus 4.6; CRQ-Mastery: 2.3 versus 2.9; all p>0.20). However, improvements in CRQ-Fatigue with pulmonary rehabilitation were greater in the COPD group (bronchiectasis 2.1 versus COPD 3.3; p=0.02).In a real-life, propensity-matched control study, patients with bronchiectasis show similar completion rates and improvements in exercise and health status outcomes as patients with COPD. This supports the routine clinical provision of pulmonary rehabilitation to patients with bronchiectasis.