Remote pulmonary rehabilitation for interstitial lung disease: developing the model using experience-based codesign.

BACKGROUND: Remote delivery may improve access to pulmonary rehabilitation (PR). Existing studies are largely limited to individuals with COPD, and the interventions have lacked codesign elements to reflect the needs and experiences of people with chronic respiratory disease, their carers/families and healthcare professionals. The aim of this study was, using experience-based codesign (EBCD), to collaborate with people with interstitial lung disease (ILD), their carers/families and healthcare professionals, to codesign a remote PR programme ready for testing in a future study. METHODS: EBCD comprises interviews, stakeholder workshops and codesign meetings. One-to-one videorecorded interviews with purposively selected people with ILD with experience of PR, their carers/families and healthcare professionals, were edited into a 20 min film. The film was shown at three audiorecorded stakeholder feedback events to identify key themes and touchpoints, and short-list key programme components. The programme was finalised at two further codesign workshops. RESULTS: Ten people with ILD, four carers/families and seven healthcare professionals were interviewed. Participants in the codesign workshops included service-user group: n=14 and healthcare professional group: n=11; joint event: n=21. Final refinements were made with small codesign teams, one comprising three people with ILD and one carer/family member, one with five healthcare professionals. The final codesigned model is a group based, supervised programme delivered by videoconference. Key elements of programme specific to ILD include recommendations to ensure participant safety in the context of desaturation risk, dedicated time for peer support and adaption of the education programme for ILD needs, including signposting to palliative care. CONCLUSION: In this EBCD project, a remote PR programme for people with ILD was codesigned by service-users, their carers/families and multidisciplinary healthcare professionals. Future research should explore the feasibility and acceptability of this intervention.

Efectos de la rehabilitación pulmonar en la capacidad funcional y calidad de vida en pacientes con enfermedad pulmonar intersticial difusa con y sin uso de oxígeno domiciliario / Effects of pulmonary rehabilitation on functional capacity and quality of life in patients with diffuse interstitial lung disease with and without use of home oxygen

Introducción La enfermedad pulmonar intersticial difusa (EPID) es un grupo de enfermedades que causan un trastorno de la capacidad aeróbica y calidad de vida, además ocasionan una gran tasa de morbimortalidad para esta población. El uso de oxigenoterapia domiciliaria tiene beneficios que aún no se comparan en programas de rehabilitación pulmonar. Objetivo Determinar los efectos de un programa de rehabilitación pulmonar en la capacidad funcional y calidad de vida relacionada con la salud en pacientes con enfermedad pulmonar intersticial difusa con y sin uso de oxígeno domiciliario. Materiales y métodos Estudio cuasiexperimental en el que todos los pacientes, por conveniencia, firmaron el consentimiento informado. Se dividieron en 2 grupos: con y sin oxígeno domiciliario, evaluados antes y después de la rehabilitación pulmonar en variables clínicas, capacidad funcional y calidad de vida. Resultados Se vincularon 31 pacientes que se dividieron en 2 grupos con oxígeno y sin oxígeno domiciliario, la mayor participación fue de hombres, edad promedio de 56 años, y se obtuvo que la mayoría había acudido a urgencias. Ambos grupos presentaron mejorías en la distancia recorrida y en la ansiedad: valor-p<0,05. La calidad de vida presentó mejorías significativas por dominio en ambos grupos: valor-p<0,05. Conclusión El grupo de EPID con uso de oxígeno domiciliario presenta mayores cambios en la capacidad funcional. En cuanto a la calidad de vida el grupo EPID sin uso de oxígeno domiciliario mejoró significativamente el dominio actividades del SGRQ, y el grupo EPID con uso de oxígeno domiciliario presentó mejoría en los dominios síntomas e impacto (AU)

Introduction Diffuse Interstitial Pulmonary Disease is a group of diseases that cause a disorder of aerobic capacity and quality of life, also cause a high rate of morbidity and mortality for this population. The use of home oxygen therapy has benefits that have not yet been compared in pulmonary rehabilitation programs. Objective To determine the effects of a pulmonary rehabilitation program on functional capacity and health-related quality of life in patients with diffuse interstitial lung disease with and without the use of home oxygen. Materials and methods Quasi-experimental study, all patients for convenience who signed the informed consent. They were divided into two groups: with and without home oxygen, evaluated before and after pulmonary rehabilitation in clinical variables, functional capacity and quality of life. Results 31 patients were linked, divided into two groups with oxygen and without home oxygen, the largest participation was men, average age 56 years, it was obtained that the majority had gone to the emergency room. Both groups presented improvements in the distance traveled and in anxiety p-value≤0.05. The quality of life presented significant improvements by domain in both groups p-value≤0.05. Conclusion The ILD group with home oxygen use presents greater changes in functional capacity. Regarding quality of life, the ILD group without home oxygen use significantly improved the activities domain of the SGRQ and the ILD group with home oxygen use showed improvement in the symptoms and impact domains (AU)

Adherencia a la rehabilitación pulmonar en pacientes con enfermedad pulmonar instersticial difusa (EPID) / Adherence to pulmonary rehabilitation in patients with diffuse interstitial pulmonary disease (DILD)

Introducción La enfermedad pulmonar intersticial difusa (EPID), a pesar de su baja prevalencia, presenta un curso progresivo y letal. Como estrategia secundaria al tratamiento farmacológico, los pacientes son remitidos a programas de rehabilitación pulmonar; no obstante, existe escasa evidencia con relación a la adherencia a dichos programas. Objetivo Analizar la adherencia de los pacientes diagnosticados con EPID a los programas de rehabilitación pulmonar en una clínica de Colombia en el año 2021. Materiales y métodos Estudio descriptivo observacional y prospectivo donde se vincularon 74 pacientes con EPID, que se dividieron en 2 grupos, adherencia baja/moderada y adherencia alta, tomando como referente el modelo de cumplimiento de Oates et al. Para la comparación de los dos grupos se realizó la prueba chi2 y la prueba T student para muestras independientes. Se tuvo en cuenta una significación del 95%, y se consideraron significativos valores p<0,05. Resultados Se obtuvo una adherencia alta en el 67,6% de los pacientes vinculados en el estudio. En las variables clínicas, diagnósticos, hospitalizaciones, días hospitalizados, y en el dominio síntomas del cuestionario de calidad de vida se presentaron diferencias significativas con un valor p≤0,05 entre los grupos de adherencia. La razón de motivo de abandono del programa de rehabilitación pulmonar en la mayoría de pacientes fue por exacerbación y no tener dinero para el transporte. Conclusiones Se presentó una alta adherencia en el 67,6% de los participantes. Pacientes con alta adherencia tenían mayor prevalencia de EPID clasificadas, hospitalizaciones, distancia recorrida y mejor calidad de vida (AU)

Introduction Diffuse interstitial lung disease (DILD), despite its low prevalence, has a progressive and lethal course. As a secondary strategy to pharmacological treatment, patients are referred to pulmonary rehabilitation programs; however, there is little evidence regarding adherence to these programs. Objective To analyze the adherence of patients diagnosed with ILD to pulmonary rehabilitation programs in a clinic in Colombia in the year 2021. Materials and methods Observational and prospective descriptive study where 74 patients with ILD were linked, who were divided into two groups, low/moderate adherence and high adherence, taking as reference the compliance model of Oates et al. For the comparison of the two groups, the chi2 test and the T student test for independent samples were performed. A significance of 95% was taken into account, and significant p-values<0.05 were considered. Results High adherence was obtained in 67.6% of the patients included in the study. In the clinical variables, diagnoses, hospitalizations, days hospitalized, and in the symptoms domain of the quality of life questionnaire, there were significant differences with a p-value≤0.05 between the adherence groups. The reason for abandonment of the pulmonary rehabilitation program in the majority of patients was due to exacerbation and not having money for transportation. Conclusions High adherence was present in 67.6% of the participants. Patients with high adherence had a higher prevalence of classified ILD, hospitalizations, distance traveled and better quality of life (AU)

Home-Based Pulmonary Rehabilitation and Health Coaching in Fibrotic Interstitial Lung Disease: IMPLEMENTATION AND QUALITATIVE ASSESSMENT OF A PILOT TELEHEALTH PROGRAM.

PURPOSE: Pulmonary rehabilitation is a behavioral modification intervention shown to improve exercise tolerance and patient-reported quality of life in patients with fibrotic interstitial lung disease. Home-based rehabilitation may provide easier access for those who struggle to complete center-based rehabilitation programs due to increased symptom burden or frailty. METHODS: We present the quantitative and qualitative findings of a pilot study of 21 patients with fibrotic interstitial lung disease who participated in a 12-wk home-based pulmonary rehabilitation program with activity monitoring and health coaching. RESULTS: Pre- and post-intervention patient-reported outcome questionnaires suggested improvements in dyspnea and respiratory-related quality of life but were underpowered to meet statistical significance. Half had increases in mean daily step counts while a quarter declined because of disease progression. Qualitative analysis of semistructured participant interviews suggested a significant baseline disease burden with related secondary impacts, including anxiety regarding disease progression and prognosis. Many who participated had no specific program expectations or self-determined goals but still found the program impactful, particularly on their abilities to adapt and cope with the disease. CONCLUSION: Our study suggests feasibility in a diverse set of patients with varying severity and diagnostic subtypes. We also provide quantitative and qualitative aspects of program impact on patient well-being and highlight the complex interaction between measured physical and self-reported outcomes and disease experience.

A Retrospective, Descriptive Study of Dyspnea Management in a Multidisciplinary Interstitial Lung Disease Clinic.

Background: Fibrotic interstitial lung diseases (F-ILDs) have a high symptom burden with progressive dyspnea as a primary feature. Breathlessness is underrecognized and undertreated primarily due to lack of consensus on how to best measure and manage it. Several nonpharmacologic and pharmacologic strategies are published in the literature, however there is a paucity of real-world data describing their systematic implementation. Objectives: We describe the types of breathlessness interventions and timing of implementation in our multidisciplinary collaborative care (MDC) ILD clinic and the impact of our approach on dyspnea trajectory and acute care use in ILD. Methods: A retrospective, observational study of deceased ILD patients seen in our clinic (2012-2018) was conducted. Patients were grouped by baseline medical research council (MRC) grade and dyspnea interventions from clinic enrolment until death were examined. Healthcare usage in the last 6 months of life was collected through Alberta's administrative database. Results: Eighty-one deceased ILD patients were identified. Self management advice was provided to 100% of patients. Pulmonary rehabilitation (PR) and home care (HC) referrals were made in 40% and 57% of patients, respectively. Eighty percent were treated with oxygen and 53% with opioids during the study. MDC-initiated referral to PR and HC, oxygen and opioid prescriptions were provided a median of 13, 9, 11, and 4 months prior to death, respectively. Stepwise implementation of interventions was observed more commonly in MRC 1-2 and concurrent implementation in MRC 4-5. Conclusions: Our clinic's approach allows early and systematic dyspnea management.

Survival after inpatient or outpatient pulmonary rehabilitation in patients with fibrotic interstitial lung disease: a multicentre retrospective cohort study.

BACKGROUND: The impact of pulmonary rehabilitation (PR) on survival in patients with fibrotic interstitial lung disease (ILD) is unknown. Given the challenges conducting a large randomised controlled trial, we aimed to determine whether improvement in 6-minute walk distance (6MWD) was associated with better survival. METHODS: This retrospective, international cohort study included patients with fibrotic ILD participating in either inpatient or outpatient PR at 12 sites in 5 countries. Multivariable models were used to estimate the association between change in 6MWD and time to death or lung transplantation accounting for clustering by centre and other confounders. RESULTS: 701 participants (445 men and 256 women) with fibrotic ILD were included. The mean±SD ages of the 196 inpatients and 505 outpatients were 70±11 and 69±12 years, respectively. Baseline/changes in 6MWD were 262±128/55±83 m for inpatients and 358±125/34±65 m for outpatients. Improvement in 6MWD during PR was associated with lower hazard rates for death or lung transplant on adjusted analysis for both inpatient (HR per 10 m 0.94, 95% CI 0.91 to 0.97, p<0.001) and outpatient PR (HR 0.97, 95% CI 0.95 to 1.00, p=0.042). Participation in ≥80% of planned outpatient PR sessions was associated with a 33% lower risk of death (95% CI 0.49% to 0.92%). CONCLUSIONS: Patients with fibrotic ILD who improved physical performance during PR had better survival compared with those who did not improve performance. Confirmation of these hypothesis-generating findings in a randomised controlled trial would be required to definitely change clinical practice, and would further support efforts to improve availability of PR for patients with fibrotic ILD.

Oxigenoterapia domiciliaria y capacidad aeróbica en pacientes con enfermedad pulmonar intersticial difusa. Estudio transversal / Home Oxygen Therapy And Aerobic Capacity In Patients With Diffuse Interstitial Lung Disease. Cross-Sectional Study

Introducción: La enfermedad pulmonar intersticial difusa es un grupo de enfermedades que causan un trastorno de la capacidad aeróbica y calidad de vida, además, ocasionan una gran tasa de morbimortalidad para esta población. El uso de oxigenoterapia domiciliaria mayor a 15 horas diarias tiene beneficios en pacientes hipoxémicos crónicos, sin embargo, poco se ha comparado su uso con pacientes que no lo reciben. Objetivo: Describir las características clínicas, capacidad aeróbica funcional y calidad de vida relacionada con la salud de dos grupos de pacientes con enfermedad pulmonar intersticial difusa, uno con indicación de oxigenoterapia domiciliaria y otro grupo sin indicación. Métodos: Estudio descriptivo transversal, que incluyó 41 pacientes con enfermedad pulmonar intersticial difusa que firmaron consentimiento informado. En ambos grupos, características demográficas y clínicas, ansiedad/depresión, calidad de vida relacionada con la salud con el cuestionario Saint George y la capacidad aeróbica funcional con la prueba de marcha de seis minutos fueron medidas. Se compararon los grupos con la prueba t de student para muestras independientes. Resultados: El grupo enfermedad pulmonar intersticial difusa con oxigenoterapia domiciliaria presentó mayor porcentaje de antecedente de tabaquismo (p = 0,041), menor distancia caminada en la prueba de marcha de seis minutos (304,1 ± 108,7 vs. 390,3 ± 95,6 p = 0,01), y menor porcentaje de la distancia predicha (58,37 ± 20,45 vs. 73,34 ± 22,90, p = 0,034) frente al grupo enfermedad pulmonar intersticial difusa sin oxigenoterapia domiciliaria. Conclusiones: Los pacientes con enfermedad pulmonar intersticial difusa con indicación de oxigenoterapia domiciliaria presentan menor capacidad aeróbica funcional comparada con pacientes sin indicación(AU)

Introduction: Diffuse interstitial lung disease is a group of diseases that cause a disorder of aerobic capacity and quality of life; in addition, they cause a high rate of morbidity and mortality for this population. The use of home oxygen therapy greater than 15 hours a day has benefits in chronic hypoxemic patients, however, little has been compared to patients who do not receive it. Objective: Describe the clinical characteristics, functional aerobic capacity and health-related quality of life of two groups of patients with diffuse interstitial lung disease, one with indication for home oxygen therapy and another group without indication. Methods: A cross-sectional descriptive study included 41 patients with diffuse interstitial lung disease who signed informed consent. In both groups, demographic and clinical characteristics, anxiety/depression, health quality of life related with the Saint George questionnaire, and functional aerobic capacity with the six-minute gait test were measured. The groups were compared with the student's t-test for independent samples. Results: The diffuse interstitial lung disease group with home oxygen therapy presented a higher percentage of smoking history (p = 0.041), a shorter distance walked in the six-minute gait test (304.1 ± 108.7 vs. 390.3 ± 95.6 p = 0.01), and a lower percentage of the predicted distance (58.37 ± 20.45 vs. 73.34 ± 22.90, p = 0.034) compared to the diffuse interstitial lung disease group without home oxygen therapy. Conclusions: Patients with diffuse interstitial lung disease with indication of home oxygen therapy have lower functional aerobic capacity compared to patients without indication(AU)

Pulmonary rehabilitation for interstitial lung disease: Referral and patient experiences.

Objectives: The objectives of this study were to determine the proportion of patients with interstitial lung disease (ILD) referred to pulmonary rehabilitation (PR) and to understand their experiences of participation or non-participation. Methods: Adults (>18 years old) with a diagnosis of ILD were identified from the Alfred Health ILD registry in Melbourne. Information regarding PR referral and attendance were collected from medical records. Semi-structured interviews with open-ended questions were conducted with patients who had been referred to PR. Results: Of 336 patients eligible for inclusion, PR referral was identified in 137 patients (40.7%). Patients referred to PR had worse respiratory function than those not referred (forced vital capacity mean 64 (SD 23) vs 79 (19) % predicted) and more desaturation during a 6-min walk test (86.6 (7.8%) vs 88.5 (7.0%)). Semi-structured interviews identified three major themes: valued components of PR (supervision and individualization, improved confidence with exercise, education and peer support); limited knowledge about PR prior to attendance and barriers to attending PR (lack of perceived benefits, fear of exercise and accessibility). Discussion: Over 40% of patients who attended a specialist ILD clinic were referred to pulmonary rehabilitation, with higher referral rates in those with more severe disease. There are opportunities to improve patient knowledge regarding the role and expected benefits of PR in people with ILD.

The effect of heated humidified nasal high flow oxygen supply on exercise tolerance in patients with interstitial lung disease: A pilot study.

BACKGROUND AND OBJECTIVES: Patients with interstitial lung disease (ILD) experience early symptoms of dyspnoea and leg fatigue during exercise together with severe and rapid oxygen desaturation. Heated and humidified nasal high flow oxygen (NHF) has been proven to enhance exercise endurance and physiological parameters in COPD patients. This study aims to evaluate the effect of NHF on exercise tolerance in ILD patients. METHODS: Twenty-five patients (10 female) with severe ILD performed three constant-load (70% maximal workload) cycling tests to exhaustion under different breathing conditions: room air, oxygen supplementation (4 L min-1 O2) and NHF (inspiratory O2 fraction 0.5, 30-50 L min-1, heated 34 °C and humidified). RESULTS: Endurance time was significantly longer with NHF (618 ± 297 s) compared to O2 (369 ± 217 s, p < 0.001) and room air (171 ± 76 s, p < 0.001). Kinetics of oxygen desaturation, chronotropic response, dyspnoea and leg fatigue sensations were delayed with NHF. At exhaustion with NHF, compared to the two other conditions, oxygen desaturation was less severe while heart rate, dyspnoea and leg fatigue were similar. CONCLUSION: NHF significantly improved endurance time, physiological parameters and sensations during exercise in severe ILD patients. NHF may be useful to improve functional capacities and facilitate pulmonary rehabilitation in ILD.

Effectiveness of Pulmonary Rehabilitation in Interstitial Lung Disease, Including Coronavirus Diseases: A Systematic Review and Meta-analysis.

OBJECTIVE: A meta-analysis of randomized controlled trials (RCTs) was conducted to determine the effect of pulmonary rehabilitation on functional capacity and quality of life in interstitial lung diseases, including those caused by coronaviruses. DATA SOURCES: MEDLINE, EMBASE, SPORTDiscus, Cochrane Library, Web of Science, and MedRxiv from inception to November 2020 were searched to identify documents. STUDY SELECTION: Publications investigating the effect of pulmonary rehabilitation on lung function (forced vital capacity [FVC]), exercise capacity (6-minute walk distance [6MWD]), health related quality of life (HRQOL), and dyspnea were searched. DATA EXTRACTION: The data were extracted into predesigned data extraction tables. Risk of bias was evaluated with the Cochrane Risk of Bias tool (RoB 2.0). DATA SYNTHESIS: A total of 11 RCTs with 637 interstitial lung disease patients were eligible for analyses. The pooled effect sizes of the association for pulmonary rehabilitation were 0.37 (95% confidence interval [CI], 0.02-0.71) for FVC, 44.55 (95% CI, 32.46-56.64) for 6MWD, 0.52 (95% CI, 0.22-0.82) for HRQOL, and 0.39 (95% CI, -0.08 to 0.87) for dyspnea. After translating these findings considering clinical improvements, pulmonary rehabilitation intervention increased predicted FVC by 5.5%, the 6MWD test improved by 44.55 m, and HRQOL improved by 3.9 points compared with baseline values. Results remained similar in sensitivity analyses. CONCLUSIONS: Although specific evidence for pulmonary rehabilitation of coronavirus disease 2019 patients has emerged, our data support that interstitial lung disease rehabilitation could be considered as an effective therapeutic strategy to improve the functional capacity and quality of life in this group of patients.

Pulmonary rehabilitation in patients with interstitial lung diseases: Correlates of success.

BACKGROUND AND AIM: Benefits of pulmonary rehabilitation in Interstitial Lung Diseases (ILD) have been reported. The aim of this large multicenter study was to identify the success predictors of pulmonary rehabilitation in a real-life setting. METHODS: Data of 240 in-patients (110 idiopathic pulmonary fibrosis (IPF), 106 ILD other than IPF and 24 undetermined ILD) undergoing pulmonary rehabilitation in a 10-year period were retrospectively evaluated. Six minute walking distance (6MWT), body weight-walking distance product tests, dyspnoea and arterial blood gases were assessed at admission and discharge. Differences in post rehabilitation changes in outcome measures as function of baseline characteristics were evaluated. RESULTS: After rehabilitation, patients showed improvements in all outcome measures (p < 0.05), regardless of the underlying diagnosis or disease severity. Patients needing oxygen therapy at rest showed reduced benefits. Baseline 6MWD inversely correlated with its changes at discharge. Non-significant greater benefits after rehabilitation were found in IPF patients under antifibrotic therapy. In a subset of 50 patients assessed on average 10.3 ± 3.5 months after discharge, the benefits in 6MWD were not maintained (312.9 ± 139.4, 369.7 ± 122.5 and 310.8 ± 139.6 m at admission, discharge and follow up respectively: p < 0.0001). CONCLUSION: Pulmonary rehabilitation may improve dyspnoea, exercise capacity and fatigue in patients with ILD of different aethiologies and level of severity. The long-term effects need to be established.

Feasibility of an online platform delivery of pulmonary rehabilitation for individuals with chronic respiratory disease.

INTRODUCTION: SARS-CoV-2 has restricted access to face-to-face delivery of pulmonary rehabilitation (PR). Evidence suggests that telehealth-PR is non-inferior to outpatient PR. However, it is unknown whether patients who have been referred to face-to-face programmes can feasibly complete an online-PR programme. METHODS: This service evaluation used a mixed-methods approach to investigate a rapid PR service remodelling using the University of Gloucestershire eLearn Moodle platform. Quantitative baseline demographic and PR outcome data were collected from online-PR participants, and semistructured interviews were completed with PR staff and participants. RESULTS: Twenty-five individuals were eligible from a PR waiting list. Thirteen declined participation and 14 completed PR. Significant pre-post online PR improvements were achieved in 1 min sit-to-stand (CI 2.1 to 9 (p=0.004)), Generalised Anxiety Disorder (CI -0.3 to -2.6 (p=0.023)), Primary Health Questionnaire-9 (CI -0.3 to -5.1 (p=0.029)), Chronic Respiratory Questionnaire dyspnoea (CI 0.5 to 1.3 (p=0.001)), fatigue (CI 0.7 to 2 (p=0.0004)), emotion (CI 0.7 to 1.7 (p=0.0002)), mastery (CI 0.4 to 1.3 (p=0.001)). Interviews indicated that patient PR inclusion was made possible with digital support and a PR introduction session improved participant engagement and safety. Incremental progression of exercise was perceived as more successful online compared with face-to-face PR. However, perceptions were that education sessions were less successful. Online-PR required significant staff time resource. DISCUSSION: Online-PR improves patient outcomes and is feasible and acceptable for individuals referred for face-to-face PR in the context of a requirement for social distancing. Face-to-face programmes can be adapted in a rapid fashion with both staff and participants perceiving benefit. Future pragmatic trials are now warranted comparing online-PR including remote assessments to centre-based PR with suitably matched outcomes, and patient and staff perceptions sought regarding barriers and facilitators of online delivery.

Effects of Vibration Training in Interstitial Lung Diseases: A Randomized Controlled Trial.

BACKGROUND: Numerous studies have reported positive effects of exercise training in patients with interstitial lung disease (ILD) on physical capacity and quality of life. However, evidence is rare on the effects of specific forms of training and further pathophysiological mechanisms in these patients. OBJECTIVES: In this multicenter study we aimed to explore the clinical effects of whole-body vibration training (WBVT) in patients with ILD on various outcome measures, including proinflammatory cytokines and myostatin. METHODS: We randomly assigned 26 patients with different forms of multidisciplinary confirmed fibrotic ILDs either to the WBVT group (n = 11; 55% male, 61 ± 14 years old, forced vital capacity 83.2 ± 29.3% predicted, 6-min walking distance [6MWD] 478 ± 79 m) performing 3 months of a standardized training (3 times per week), or to a control training group (CTG, n = 15; 60% male, 63 ± 9 years old, FVC 74.6 ± 20.5% predicted, 6MWD 455 ± 85 m) performing sham WBV training. Training in the two groups was performed on a GalileoTM vibration plate (6-20 vs. 5 Hz). The functional assessments before and after the intervention period included pulmonary function, 6MWD test, chair rise test, ultrasonographic measurement of quadriceps muscle thickness (cross-sectional area), quality of life questionnaires, and serum samples. RESULTS: We observed a significant increase in 6MWD (∆Training = 30 m [12-67], p = 0.024) and a decrease of myostatin (∆Training = -465 pg/mL [-713 to -166], p = 0.008) in the WBVT group. In contrast, no significant differences were observed in the CTG. CONCLUSIONS: The present study demonstrates that WBVT is able to significantly increase 6MWD and decrease myostatin in patients with fibrotic ILDs. Therefore, WBVT seems to be a beneficial and feasible training modality in ILD patients. Clinical Trial Registry: German Clinical Trials Registry (DRKS00012930).

Pulmonary rehabilitation in interstitial lung diseases.

PURPOSE OF REVIEW: This review will provide an overview of the components and current evidence to support pulmonary rehabilitation in patients with interstitial lung diseases (ILD), focusing on the latest findings in enhancing quality of life and functional capacity using exercise training and education. RECENT FINDINGS: Recent literature demonstrates a longer duration of pulmonary rehabilitation results in sustained improvements in functional capacity and quality of life in patients with ILD. Educational components tailored to ILD provide added value to supervised exercise training. Enhancing exercise training with hyperoxia merits further study and other modalities such as pursed-lip breathing have been shown to be counterproductive. SUMMARY: Recent evidence continues to support the use of tailored pulmonary rehabilitation programs in patients with ILD. Areas which require further investigation include identifying optimal exercise training regimens, educational topics, and breathing techniques that are specifically designed for ILD.

COVID-19 rapid guideline: interstitial lung disease

The purpose of this guideline is to maximise the safety of adults with interstitial lung disease, including idiopathic pulmonary fibrosis and pulmonary sarcoidosis, during the COVID-19 pandemic. It also aims to protect staff from infection and enable services to make the best use of NHS resources.

Ambulatory Oxygen in Fibrotic Interstitial Lung Disease: A Pilot, Randomized, Triple-Blinded, Sham-Controlled Trial.

BACKGROUND: Despite a lack of evidence, ambulatory oxygen therapy is frequently prescribed for patients with interstitial lung disease (ILD) and exertional desaturation. Patients often prefer portable oxygen concentrators to oxygen cylinders. This study aimed to examine the feasibility of conducting a clinical trial of ambulatory oxygen delivered via portable concentrators in patients with ILD. RESEARCH QUESTION: Is it feasible to conduct a clinical trial of ambulatory oxygen delivered via portable concentrators in patients with ILD? STUDY DESIGN AND METHODS: In this randomized, triple-blinded, sham-controlled trial, 30 participants with ILD and isolated exertional desaturation to < 90% on 6-minute walk tests were randomized to 12-week ambulatory oxygen or air delivered via portable concentrators, with assessments performed at baseline and weeks 4, 12, and 18. Primary outcomes were trial feasibility and the change in 6-minute walk distance (6MWD) on room air at week 12. RESULTS: Study recruitment was completed within 18 months, with six withdrawals. Participant blinding was successful, with the Bang's Blinding Index being 0 (95% CI, -0.40 to 0.40) for the oxygen group and 0 (95% CI, -0.42 to 0.42) for the sham group. No significant difference in 6MWD was seen between groups at week 12 (mean difference of -34 m [95% CI, -105 to 36], P = .34). For secondary outcomes, compared with the sham group, the oxygen group had a significantly higher Leicester Cough Questionnaire psychological domain score, indicating better cough-related quality of life (mean difference of 0.9 [95% CI, 0.2 to 1.6], P = .01), but a shorter duration of moderate-to-vigorous activities (mean difference of -135 [95% CI, -267 to -3], P = .04) at week 12. INTERPRETATION: Based on the results of this pilot study, a definitive randomized controlled trial with a longer intervention duration is warranted to clarify therapeutic impacts of ambulatory oxygen in patients with ILD. TRIAL REGISTRY: Australian New Zealand Clinical Trials Registry; No.: ACTRN12617000054314; URL: www.anzctr.org.au/.

Pulmonary rehabilitation in interstitial lung diseases: A review of the literature.

There are more than 200 different diseases classed as interstitial lung diseases (ILDs). For epidemiological and practical purposes, ILDs are classified into diseases of known and unknown etiology. The aim of this review is to evaluate our current knowledge about the efficacy and safety of pulmonary rehabilitation (PR) in patients with ILDs. Other issues, such as ILD pathogenesis, prevalence and comorbidity, are also elaborated in the review. Pulmonary rehabilitation is an important part of comprehensive care for patients with ILDs. In comparison to PR for patients with chronic pulmonary obstructive disease (COPD), the number of clinical studies concerning PR for patients with ILDs is small. The majority of trials have been performed in relatively small groups of patients. The principles of PR in this group of patients are the same as for patients with COPD. Exercise-induced desaturation is frequently observed during PR, which is the main source of complications in patients with ILDs. Major differences between ILD and COPD patients include poorer exercise tolerance and faster development of respiratory failure in patients with ILDs.

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.