Pulmonary rehabilitation in idiopathic pulmonary fibrosis.

PURPOSE OF REVIEW: This review synthesizes the expanding evidence for pulmonary rehabilitation that has led to its recommended inclusion in the holistic care of people with idiopathic pulmonary fibrosis (IPF), as well as discussing strategies that may maximize and sustain benefits. RECENT FINDINGS: Pulmonary rehabilitation is an effective intervention leading to significant improvements in exercise tolerance, symptoms, and quality of life for people with IPF. Improvements in symptoms and quality of life can persist longer term, whereas functional capacity does not; therefore, strategies to preserve functional capacity are an important area of research. Referral early in the disease course is encouraged to promote longer lasting effects. Evidence that high-intensity interval training may optimize benefits of exercise training is emerging. Supplemental oxygen is frequently used to manage exercise-induced desaturation, although its use as an adjunct therapy requires more evidence. SUMMARY: Current evidence strongly supports the inclusion of pulmonary rehabilitation in the standard holistic care of IPF, with early participation encouraged. Further research is needed to establish the optimal exercise strategies, modalities and adjunct therapies that enhance outcomes of pulmonary rehabilitation and promote longer lasting effects.

Understanding the lived experience of idiopathic pulmonary fibrosis and how this shapes views on home-based pulmonary rehabilitation in Delhi, India.

OBJECTIVES: Pulmonary Rehabilitation (PR) is a high-impact intervention for individuals with idiopathic pulmonary fibrosis (IPF) but access is limited in India. PR barriers include distance to travel, lack of service provision and lack of healthcare professionals to deliver PR, thus it is disproportionate to the immense burden of IPF in India. We explored the lived experiences of people living with IPF, family caregivers (CGs) and healthcare workers (HCWs) as well as their views towards home-based PR (HBPR) in Delhi, India. METHODS: A qualitative study using semi-structured interviews with individuals with IPF (n = 20), CGs (n = 10) and HCWs (n = 10) was conducted. Data were analysed using codebook thematic analysis. RESULTS: Three major themes were generated: (i) Health impact, which included pathophysiological changes, range of symptoms experienced, disease consequences and impact of comorbidities; (ii) Disease management, which described strategies to control the progression and overall management of IPF, such as medications and exercises; (iii) Mode of Pulmonary Rehabilitation, which described perceptions regarding HBPR, comparisons with centre-based programmes, and how HBPR may fit as part of a menu of PR delivery options. CONCLUSION: People living with IPF, family caregivers and healthcare workers were positive about the potential implementation of HBPR and suggested the development of a paper-based manual to facilitate HBPR over digital/online approaches. The content of HBPR should be sensitive to the additional impact of non-IPF health issues and challenges of reduced interactions with healthcare professionals.

Changes in physical activity in people with idiopathic pulmonary fibrosis before and after virtual pulmonary rehabilitation: a feasibility study.

BACKGROUND: Pulmonary rehabilitation (PR) is recommended for the treatment of people with idiopathic pulmonary fibrosis (IPF). Physical activity is an important health behaviour, closely linked to survival in people with IPF. Little is known about the impact of virtual (V) PR on physical activity in people with IPF. OBJECTIVE: To explore the feasibility of conducting a trial to explore effect of virtual PR on objectively measured physical activity in people with IPF. METHODS: All patients with a diagnosis of IPF in a stable phase of the disease were invited to participate in VPR: a 10 week exercise programme delivered twice-weekly for one hour. Data were collected at baseline (BL) and post VPR (10 weeks): Kings Brief Interstitial Lung Disease (K-BILD), Exercise capacity (6-minute walk test (6MWT) or 1-minute sit-to-stand (STS)) and Physical Activity. Physical activity was measured with a triaxial accelerometer for seven days. Screening, recruitment, adherence and safety data were collected. RESULTS: 68 people were screened for this study. N = 16 participants were recruited to the study. There was one dropout. N = 15 completed VPR. All results reported in mean (standard deviation) (SD). Participants attended 18.1(2.0) of the 20 sessions. No adverse events were detected. The mean age of participants was 71.5(11.5) years, range: 47-95 years; 7 M:9 F. Mean (SD) FEV1 2.3(0.3)L, FVC 2.8(0.7)L. No statistically significant changes were observed in outcome measures apart from exercise capacity. Light physical activity increased from 152(69.4) minutes per day (n = 16) to 161.9(88.7) minutes per day (n = 14), mean change (SD) (CI) p-value: 9.9 (39.8) [-12.3 to 30.9] p = 0.4. Moderate-to-vigorous physical activity increased from 19.1(18.6) minutes per day (n = 16) to 25.7(28.3) minutes per day (n = 14), mean change (SD) (CI) p-value: 6.7 (15.5) [-2.1 to 15.1] p = 0.1. Step count increased from 3838(2847) steps per day (n = 16) to 4537(3748) steps per day (n = 14), mean change (SD) (CI) p-value: 738 (1916) [-419.3 to 1734.6] p = 0.2. K-BILD (n = 15) increased from 55.1(7.4) at BL to 55.7(7.9) post VPR mean change (SD) [95% confidence interval] (CI) p-value: 1.7(6.5) [-1.7 to 5.3], p = 0.3. 6MWT (n = 5) increased from 361.5(127.1) to 452.2(136.1) meters, mean change (SD) (CI) p-value: 63.7 (48.2) [-3.8 to 123.6], p = 0.04 and 1-minute STS increased from 17.6(3.0) (n = 11) to 23.7(6.3) (n = 10), mean change (SD) (CI) p-value 5.8 (4.6) [2.6 to 9.1], p = 0.003. CONCLUSION: VPR can improve physical activity in people with IPF. A number of important feasibility issues included recruitment, retention, adherence and safety have been reported which are crucial for future research in this area. A fully powered trial is needed to determine the response of people with IPF to PR with regard to physical activity.

Clinical evidence for improving exercise tolerance and quality of life with pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis: A systematic review and meta-analysis.

OBJECTIVE: To evaluate the efficacy and safety of pulmonary rehabilitation for exercise tolerance and quality of life improvement in idiopathic pulmonary fibrosis. METHODS: We searched PubMed, Cochrane Library, Embase, Web of Science, Chinese Biomedical Literature Database, China National Knowledge Infrastructure, Wanfang Database, and Chongqing VIP for randomized controlled trials that compared pulmonary rehabilitation with usual care for idiopathic pulmonary fibrosis. The risk of bias and certainty of evidence were assessed using Cochrane Collaboration's Risk of Bias tool and the Grading of Recommendations, Assessment, Development and Evaluation criteria. RESULTS: Eleven trials in total with 549 participants. Compared with usual care, pulmonary rehabilitation significantly increased 6-minute walking distance (mean difference: 35.2m, 95% confidence intervals: 25.4m-44.9m; ten trials; 447 participants; moderate), decreased the St George's Respiratory Questionnaire total scores (mean difference: -9.11, 95% confidence intervals: -10.78 to -7.43; six trials; 303 participants; moderate), and reduced the modified Medical Research Council scores were lower (mean difference: -0.76, 95% confidence intervals: -1.25 to -0.27; three trials; 196 participants; low). Improvements were noted in forced vital capacity percent-predicted (mean difference: 4.88, 95% confidence intervals: 2.67 to 7.10; four trials; 214 participants; moderate) and diffusing capacity for carbon monoxide (mean difference: 4.71, 95% confidence intervals: 0.96 to 8.46; six trials; 358 participants; low). CONCLUSIONS: Pulmonary rehabilitation may significantly improve exercise tolerance and quality of life in idiopathic pulmonary fibrosis patients, but the quality of evidence was low to moderate. Large sample, multicenter, randomized controlled trials are needed to verify the efficacy and safety of pulmonary rehabilitation.

Evolution and treatment of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic and devastating disease of unknown etiology, characterized by irreversible morphological changes, ultimately leading to lung fibrosis and death. In recent years, significant progress has been achieved in understanding the pathogenesis of IPF. Moreover, we assisted to the conceptual change of the pathogenic hypothesis that currently considers IPF as a primarily fibrotic driven disease. However, despite the undeniable progress, the diagnosis of IPF remains still very complex requiring the presence of a team of experts to achieve the highest level of diagnostic confidence. The advent of antifibrotics has radically changed the treatment landscape of IPF and new promising drugs are currently under evaluation. Furthermore, a more extensive use of non-pharmacological treatments has also to be encouraged in all patients both to reduce symptoms and improve quality of life.

Home-Based Pulmonary Rehabilitation for Patients With Idiopathic Pulmonary Fibrosis: A PILOT STUDY.

PURPOSE: To evaluate the adherence and effectiveness of a home-based exergame program for patients with idiopathic pulmonary fibrosis (IPF). METHOD: Patients with IPF were randomly assigned to a relatively unsupervised Wii Fit exergame intervention group or Wii video game control group (with no active whole-body movement involved). Participants in both groups were instructed to play their respective games 30 min/d, 3 d/wk for 12 wk. In addition, they were asked to perform their usual exercise/physical activities. Outcome measures were 6-min walk distance (6MWD), exercise-related dyspnea, and St George's Respiratory Questionnaire (SGRQ). RESULTS: The 20 participants differed significantly between intervention and control groups in baseline characteristics (forced vital capacity = 2.0 ± 0.5 vs 3.1 ± 0.7 L; forced expiratory volume in 1 sec = 1.7 ± 0.4 vs 2.5 ± 0.6 L, respectively). Participant adherence rate to the exergame program was very low (20%). There was no significant improvement in the outcome measures in either group. In fact, both the intervention and control groups had a deterioration in 6MWD (-22 ± 56 m vs -60 ± 111 m), respectively and SGRQ scores (3 ± 9 vs 1 ± 11), respectively. CONCLUSIONS: The home-based exergame intervention for patients with IPF did not show improvement in functional performance, dyspnea, or health-related quality of life at the completion of the 12-wk program in our 2 heterogeneous groups. In addition to the low adherence rate, insufficient frequencies and durations of exergaming may contribute to the lack of improvement. A lack of effectiveness of home-based pulmonary rehabilitation using exergaming for patients with IPF appears consistent with prior observational studies that used more traditional modes of home-based exercise.

Pulmonary rehabilitation improves survival in patients with idiopathic pulmonary fibrosis undergoing lung transplantation.

This study was conducted to evaluate whether a pulmonary rehabilitation program (PRP) is independently associated with survival in patients with idiopathic pulmonary fibrosis (IPF) undergoing lung transplant (LTx). This quasi-experimental study included 89 patients who underwent LTx due to IPF. Thirty-two completed all 36 sessions in a PRP while on the waiting list for LTx (PRP group), and 53 completed fewer than 36 sessions (controls). Survival after LTx was the main outcome; invasive mechanical ventilation (IMV), length of stay (LOS) in intensive care unit (ICU) and in hospital were secondary outcomes. Kaplan-Meier curves and Cox regression models were used in survival analyses. Cox regression models showed that the PRP group had a reduced 54.0% (hazard ratio = 0.464, 95% confidence interval 0.222-0.970, p = 0.041) risk of death. A lower number of patients in the PRP group required IMV for more than 24 hours after LTx (9.0% vs. 41.6% p = 0.001). This group also spent a mean of 5 days less in the ICU (p = 0.004) and 5 days less in hospital (p = 0.046). In conclusion, PRP PRP completion halved the risk of cumulative mortality in patients with IPF undergoing unilateral LTx.

Pulmonary Rehabilitation for Exercise Tolerance and Quality of Life in IPF Patients: A Systematic Review and Meta-Analysis.

OBJECTIVE: The aim of this study is to evaluate the efficacy and safety of pulmonary rehabilitation (PR) in patients with idiopathic pulmonary fibrosis (IPF). Methods. Embase, PubMed, Cochrane Library, China National Knowledge Infrastructure (CNKI), Chongqing VIP (CQVIP), Wanfang Data, and Chinese Biomedical Literature Database (SinoMed) were comprehensively searched. Randomized controlled trials (RCTs) that investigated the effects of PR for IPF patients were included. Literature selection and data extraction were conducted by two review authors independently. The Cochrane Collaboration's Risk of Bias tool and RevMan software (version 5.3) were used to evaluate the quality of studies and conduct statistical analysis, respectively. Results. Seven studies (190 participants) were included. PR had a significant effect on six-minute walk distance (6MWD) (MD:48.60; 95%CI: 29.03 to 68.18; Z=4.87, P<0.00001), and 6MWD was improved more in subgroup analysis including studies conducted in Asia (MD: 53.62; 95%CI: 30.48 to 76.66; Z=4.54, P<0.00001) and Europe (MD:54.10; 95% CI: 26.65 to 101.56; Z=2.23, P=0.03). Forced vital capacity (FVC%) was higher (MD: 3.69; 95%CI: 0.16 to 7.23; Z=2.05, P=0.04). St. George's Respiratory Questionnaire (SGRQ)/IPF-specific SGRQ (SGRQ-I) total score was lower (MD: -7.87; 95% CI: -11.44 to -4.30; Z=4.32, P<0.0001). No significant effects were found for lung diffusing capacity determined by the single-breath technique (DLCO%) (MD: 3.02; 95%CI: -0.38 to 6.42; Z=1.74, P=0.08). CONCLUSIONS: This study suggests that PR may enhance exercise capacity and improve quality of life in IPF patients. Besides, PR may also delay the decline of lung function of patients with IPF. However, further research should more fully assess the efficacy and safety of PR for IPF.

Cardiorespiratory adaptation during 6-Minute Walk Test in fibrotic idiopathic interstitial pneumonia patients who did or did not respond to pulmonary rehabilitation.

BACKGROUND: Pulmonary rehabilitation (PR) improves performance in the 6-min walk test (6MWT) in a subset of patients with fibrotic idiopathic interstitial pneumonia (f-IIP); however, a large proportion of patients does not respond to PR. AIM: To investigate the effects of a PR program on cardiorespiratory responses during a 6MWT and to identify the characteristics of patients who do not show improved performance after PR. DESIGN: An observational study. SETTING: Patients were recruited from the Competence Centre for Rare Pulmonary Diseases at Lille University Hospital, France and completed an 8-week home-based PR program. POPULATION: A total of 19 patients with f-IIP; 12 with idiopathic pulmonary fibrosis (IPF) and 7 with fibrotic non-specific interstitial pneumonia. METHODS: Patients underwent spirometry and completed a 6MWT before and after an 8-week PR program. Gas exchange, heart rate, and pulse O2 saturation were measured continuously during the 6MWT. Quality of life, dyspnea, and anxiety/depression were assessed using the Short-Form 36 (SF-36), the baseline/transition dyspnea index (BDI/TDI), and the Hospital Anxiety and Depression Scale (HADS) questionnaires. RESULTS: Patients who did and did not improve the distance walked in the 6MWT by at least 30 m after PR were classified as responders (N.=9) and non-responders (N.=10), respectively. O2 uptake, ventilation rate, and distance covered during the 6MWT were significantly improved only in the responder group (P<0.05). Changes in SF-36, BDI/TDI, and HADS scores did not differ significantly between responders and non-responders. The non-responder group contained significantly more patients with IPF (P<0.05) and experienced greater arterial oxygen desaturation during the 6MWT compared with the responder group. CONCLUSIONS: Failure to improve performance in the 6MWT after PR was associated with a diagnosis of IPF, non-improvement in gas exchange, and greater arterial oxygen desaturation. CLINICAL REHABILITATION IMPACT: Most f-IIP patients who did not respond to PR were diagnosed with IPF and displayed greater hypoxemia during exercise. Clinical practitioners should seek to determine why patients fail to improve exercise performance after PR and propose an alternative exercise regimen to these patients.

Short- and Long-Term Reliability of the 6-Minute Walk Test in People With Idiopathic Pulmonary Fibrosis.

BACKGROUND: The aim of this work was to investigate the short- and long-term test-retest reliability of the 6-min walk distance (6MWD), peak heart rate, and nadir oxygen desaturation in idiopathic pulmonary fibrosis (IPF). METHODS: A reliability study of 70 adults with IPF was undertaken within out-patient pulmonary rehabilitation programs at 2 tertiary hospitals. Participants completed 2 baseline 6-min walk tests using a standard protocol, with continuous measures of percutaneous SpO2 and heart rate via pulse oximetry. The 6-min walk test was completed immediately following an intervention period and 6 months after. Reproducibility was assessed by intraclass correlation coefficient and Bland-Altman analysis. RESULTS: Participants with a mean ± SD diffusing capacity of the lung for carbon monoxide of 48 ± 14% were included. The reliability of the 6MWD was high (intraclass correlation coefficient = 0.96) with a mean learning effect of 21 m (95% CI 12-30 m). The learning effect persisted at 8 weeks (mean 14 m, 95% CI 5-23 m) but not 6 months (mean 15 m, 95% CI -1 to 30 m). Using the best (greatest) 6MWD significantly reduced the proportion of participants who were classified as having a clinically important response to rehabilitation compared with using the first 6MWD (40% vs 54%, P = .002). Nadir SpO2 was reproducible, with a mean difference of 0.7 ± 2.2%, and limits of agreement of -4 to 5%. Peak heart rate was more variable, with mean difference 5 ± 9 beats/min and limits of agreement of -12 to 20 beats/min. CONCLUSIONS: The 6MWD is a reproducible measure of exercise capacity in people with IPF. Whereas the nadir SpO2 may be accurately determined from one test, evaluating change in 6MWD with interventions may require 2 tests on each occasion. (ClinicalTrials.gov registration NCT0016828.).

Short- and long-term effects of pulmonary rehabilitation for idiopathic pulmonary fibrosis: a systematic review and meta-analysis.

OBJECTIVE: To investigate the short- and long-term effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis (IPF). DATA SOURCES: An electronic search of MEDLINE, Embase and Cochrane Central databases along with hand search of relevant papers were performed on 15 March 2018. REVIEW METHODS: This study was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We searched the literature for randomized controlled trials of pulmonary rehabilitation in IPF patients. The outcomes were exercise capacity and health-related quality of life. Two authors independently extracted data, assessed trial eligibility and risk of bias. Meta-analyses were performed using RevMan and STATA software. RESULTS: We extracted data from four randomized controlled trials (142 participants). At short-term follow-up, pulmonary rehabilitation significantly enhanced 6-minute walk distance (6-MWD; weighted mean difference (WMD) = 38.38, 95% confidence interval (CI) = 4.64-72.12, I2 = 60.7%; P < 0.05), reduced St. George's Respiratory Questionnaire (SGRQ)/IPF-specific SGRQ (SGRQ-I) total score (WMD = -8.40, 95% CI = -11.44 to -5.36, I2 = 0%; P < 0.00001). At long-term follow-up, pulmonary rehabilitation could not enhance 6-MWD (WMD = 17.02, 95% CI = -26.87 to 60.81, I2 = 36.3%; P = 0.43) or reduce SGRQ/SGRQ-I total score (WMD = -3.45, 95% CI = -8.55 to 1.64, I2 = 38.3%; P = 0.088). CONCLUSION: In patients with IPF, pulmonary rehabilitation showed short-term effects in enhancing exercise capacity and health-related quality of life, while it had no detectable effects at long-term follow-up.

Benefits of Pulmonary Rehabilitation in Idiopathic Pulmonary Fibrosis: A CASE REPORT.

CLINICAL CASE: We describe the case of a 50-y-old man with idiopathic pulmonary fibrosis (IPF) who underwent pulmonary rehabilitation (PR). The 8-wk training program, including resistance training and aerobic exercises, was conducted 3 d/wk under physiotherapist supervision. Clinical and functional assessment was performed prior to and following the program. At the end of the training program, meaningful improvements in primary outcomes, including spirometry values and exertional parameters, were noted. DISCUSSION: Pulmonary rehabilitation may represent a valid treatment in the management of symptoms in patients with IPF. Although the current guidelines for diagnosis and management of IPF recommend the use of PR programs, patients are not routinely referred to PR centers and exercise training for these patients is not standardized. SUMMARY: Idiopathic pulmonary fibrosis is a progressive and fatal disease characterized by the loss of lung function, which results in a severe impairment of daily activities. Prospective studies testing the effectiveness of PR programs in larger cohorts of patients are still lacking. Furthermore, a standardization of pulmonary training programs should be developed to better understand the benefit of PR.

Pulmonary Rehabilitation in Patients With Advanced Idiopathic Pulmonary Fibrosis Referred for Lung Transplantation.

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.

Impact of Pulmonary Rehabilitation on Exercise Tolerance and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A SYSTEMATIC REVIEW AND META-ANALYSIS.

BACKGROUND: Patients with pulmonary fibrosis are living longer and present with an increasing number of comorbidities over time. Pulmonary rehabilitation, as a nonpharmacological approach, may be promising in these patients, although there is limited information on the impact of pulmonary rehabilitation on exercise tolerance and quality of life. Thus, conducting a systematic review and meta-analysis, the purpose of this study was to determine the effects of pulmonary rehabilitation on exercise tolerance and quality of life in patients with idiopathic pulmonary fibrosis. METHODS: We searched MEDLINE, Cochrane Library, Embase, Scielo, PEDro, and CINAHL (from the earliest date available to June 2016) for trials. Study selection included randomized controlled trials (RCTs) that examined the effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Two reviewers selected studies independently. Data were extracted from published RCTs. Study quality was evaluated using the PEDro scale. Weighted mean differences, standard mean differences, and 95% CIs were calculated. RESULTS: We analyzed data from 5 RCTs comparing a pulmonary rehabilitation group with a control group. Pulmonary rehabilitation improved exercise tolerance weighted mean differences (44 m; 95% CI, 5.3-82.8) compared with no exercise. The meta-analyses also showed significant improvement in symptoms, impact, and total score from the St George's Respiratory Questionnaire for participants in pulmonary rehabilitation compared with control. No serious adverse events were reported. CONCLUSION: Pulmonary rehabilitation is effective in increasing exercise tolerance and improving quality of life in patients with idiopathic pulmonary fibrosis.

Phenotypic characteristics associated with slow gait speed in idiopathic pulmonary fibrosis.

BACKGROUND AND OBJECTIVE: Usual gait speed over 4 m (4MGS) is an established functional performance measure in older adults that consistently predicts adverse health outcomes, but few data exist in idiopathic pulmonary fibrosis (IPF). We assessed the reliability of 4MGS, its relationship with established outcome measures and its responsiveness to pulmonary rehabilitation. METHODS: In four prospective IPF cohorts, 4MGS inter-observer (n = 46) and test-retest (n = 46) reliability, concurrent validity (n = 65 and n = 62) and responsiveness (n = 60) were determined. The phenotypic characteristics of all patients stratified according to slow 4MGS (<0.8 m/s) were compared, including lung function parameters, HRCT of the chest, 6-min walking distance (6MWD), Medical Respiratory Council (MRC) dyspnoea score, King's Brief Interstitial Lung Disease (KBILD) questionnaire and Gender, Age and lung Physiology (GAP) prognostic index. RESULTS: Intra-class correlation coefficients for inter-observer and test-retest reliability were 0.996 and 0.983, respectively. There was a strong association between 4MGS and 6MWD (r = 0.76; P < 0.0001) and moderate correlations with MRC (r = -0.56), KBILD (r = 0.44) and GAP index (r = -0.41); all P < 0.005. 4MGS improved significantly with pulmonary rehabilitation (mean (95% CI) change: 0.16 (0.12-0.20) m/s), effect size 0.65. Patients with slow 4MGS had significantly worse exercise performance (6MWD: -167 (-220 to -133) m), dyspnoea, health status and prognosis index than those with preserved 4MGS, despite similar lung function and HRCT parameters. CONCLUSION: 4MGS is a simple, reliable, valid and responsive tool that may detect a patient phenotype with worse exercise performance, dyspnoea, health status and prognosis index in stable IPF.

Physical activity and activity space in patients with pulmonary fibrosis not prescribed supplemental oxygen.

BACKGROUND: Patients with pulmonary fibrosis (PF) have impaired quality of life, and research suggests that dyspnea and physical activity are primary drivers. As PF progresses, some patients notice the disease "shrinks their worlds". The objective of this study is to describe movement (both physical activity and activity space) in a cohort of patients with PF of various etiologies who have not been prescribed supplemental oxygen (O2). METHODS: Subjects with PF not on supplemental O2 during the day were enrolled from across the U.S. from August 2013 to October 2015. At enrollment, each subject completed questionnaires and, for seven consecutive days, wore an accelerometer and GPS tracker. RESULTS: One hundred ninety-four subjects had a confirmed diagnosis of PF and complete, analyzable GPS data. The cohort was predominantly male (56%), Caucasian (95%) and had idiopathic pulmonary fibrosis (30%) or connective tissue disease related-PF (31%). Subjects walked a median 7497 (interquartile range [IQR] 5766-9261) steps per day. Steps per day were correlated with symptoms and several quality of life domains. In a model controlling for age, body mass index, wrist- (vs. waist) worn accelerometer and percent predicted diffusing capacity (DLCO%), fatigue (beta coefficient = -51.5 ± 11.7, p < 0.0001) was an independent predictor of steps per day (model R2=0.34). CONCLUSIONS: Patients with PF, who have not been prescribed O2 for use during the day, have wide variability in their mobility. Day-to-day physical activity is related to several domains that impact quality of life, but GPS-derived activity space is not. Wearable data collection devices may be used to determine whether and how therapeutic interventions impact movement in PF patients. TRIAL REGISTRATION: NCT01961362 . Registered 9 October, 2013.

The evidence of benefits of exercise training in interstitial lung disease: a randomised controlled trial.

BACKGROUND: Uncertainty exists regarding the clinical relevance of exercise training across the range of interstitial lung diseases (ILDs). OBJECTIVE: To establish the impact of exercise training in patients with ILDs of differing aetiology and severity. METHODS: 142 participants with ILD (61 idiopathic pulmonary fibrosis (IPF), 22 asbestosis, 23 connective tissue disease-related ILD (CTD-ILD) and 36 with other aetiologies) were randomised to either 8 weeks of supervised exercise training or usual care. Six-minute walk distance (6MWD), Chronic Respiratory Disease Questionnaire (CRDQ), St George Respiratory Questionnaire IPF-specific version (SGRQ-I) and modified Medical Research Council dyspnoea score were measured at baseline, 9 weeks and 6 months. MEASUREMENTS AND MAIN RESULTS: Exercise training significantly increased 6MWD (25 m, 95% CI 2 to 47 m) and health-related quality of life (CRDQ and SGRQ-I) in people with ILD. Larger improvements in 6MWD, CRDQ, SGRQ-I and dyspnoea occurred in asbestosis and IPF compared with CTD-ILD, but with few significant differences between subgroups. Benefits declined at 6 months except in CTD-ILD. Lower baseline 6MWD and worse baseline symptoms were associated with greater benefit in 6MWD and symptoms following training. Greater gains were seen in those whose exercise prescription was successfully progressed according to the protocol. At 6 months, sustained improvements in 6MWD and symptoms were associated with better baseline lung function and less pulmonary hypertension. CONCLUSIONS: Exercise training is effective in patients across the range of ILDs, with clinically meaningful benefits in asbestosis and IPF. Successful exercise progression maximises improvements and sustained treatment effects favour those with milder disease. TRIAL REGISTRATION NUMBER: Results, ACTRN12611000416998.