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.

Diagnosis and management of connective tissue disease-associated interstitial lung disease in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand.

Pulmonary complications in CTD are common and can involve the interstitium, airways, pleura and pulmonary vasculature. ILD can occur in all CTD (CTD-ILD), and may vary from limited, non-progressive lung involvement, to fulminant, life-threatening disease. Given the potential for major adverse outcomes in CTD-ILD, accurate diagnosis, assessment and careful consideration of therapeutic intervention are a priority. Limited data are available to guide management decisions in CTD-ILD. Autoimmune-mediated pulmonary inflammation is considered a key pathobiological pathway in these disorders, and immunosuppressive therapy is generally regarded the cornerstone of treatment for severe and/or progressive CTD-ILD. However, the natural history of CTD-ILD in individual patients can be difficult to predict, and deciding who to treat, when and with what agent can be challenging. Establishing realistic therapeutic goals from both the patient and clinician perspective requires considerable expertise. The document aims to provide a framework for clinicians to aid in the assessment and management of ILD in the major CTD. A suggested approach to diagnosis and monitoring of CTD-ILD and, where available, evidence-based, disease-specific approaches to treatment have been provided.

Pulmonary rehabilitation for interstitial lung disease.

BACKGROUND: Interstitial lung disease (ILD) is characterised by reduced functional capacity, dyspnoea and exercise-induced hypoxia. Pulmonary rehabilitation is often used to improve symptoms, health-related quality of life and functional status in other chronic lung conditions. There is accumulating evidence for comparable effects of pulmonary rehabilitation in people with ILD. However, further information is needed to clarify the long-term benefit and to strengthen the rationale for pulmonary rehabilitation to be incorporated into standard clinical management of people with ILD. This review updates the results reported in 2014. OBJECTIVES: To determine whether pulmonary rehabilitation in people with ILD has beneficial effects on exercise capacity, symptoms, quality of life and survival compared with no pulmonary rehabilitation in people with ILD. To assess the safety of pulmonary rehabilitation in people with ILD. SEARCH METHODS: We searched CENTRAL, MEDLINE (Ovid), Embase (Ovid), CINAHL (EBSCO) and PEDro from inception to April 2020. We searched the reference lists of relevant studies, international clinical trial registries and respiratory conference abstracts to look for qualifying studies. SELECTION CRITERIA: We included randomised controlled trials and quasi-randomised controlled trials in which pulmonary rehabilitation was compared with no pulmonary rehabilitation or with other therapy in people with ILD of any origin. DATA COLLECTION AND ANALYSIS: Two review authors independently selected trials for inclusion, extracted data and assessed risk of bias. We contacted study authors to request missing data and information regarding adverse effects. We specified a priori subgroup analyses for participants with idiopathic pulmonary fibrosis (IPF) and participants with severe lung disease (low diffusing capacity or desaturation during exercise). There were insufficient data to perform the prespecified subgroup analysis for type of exercise training modality. MAIN RESULTS: For this update, we included an additional 12 studies resulting in a total of 21 studies. We included 16 studies in the meta-analysis (356 participants undertook pulmonary rehabilitation and 319 were control participants). The mean age of participants ranged from 36 to 72 years and included people with ILD of varying aetiology, sarcoidosis or IPF (with mean transfer factor of carbon dioxide (TLCO) % predicted ranging from 37% to 63%). Most pulmonary rehabilitation programmes were conducted in an outpatient setting, with a small number conducted in home-based, inpatient or tele-rehabilitation settings. The duration of pulmonary rehabilitation ranged from three to 48 weeks. There was a moderate risk of bias due to the absence of outcome assessor blinding and intention-to-treat analyses and the inadequate reporting of randomisation and allocation procedures in 60% of the studies. Pulmonary rehabilitation probably improves the six-minute walk distance (6MWD) with mean difference (MD) of 40.07 metres, 95% confidence interval (CI) 32.70 to 47.44; 585 participants; moderate-certainty evidence). There may be improvements in peak workload (MD 9.04 watts, 95% CI 6.07 to 12.0; 159 participants; low-certainty evidence), peak oxygen consumption (MD 1.28 mL/kg/minute, 95% CI 0.51 to 2.05; 94 participants; low-certainty evidence) and maximum ventilation (MD 7.21 L/minute, 95% CI 4.10 to 10.32; 94 participants; low-certainty evidence). In the subgroup of participants with IPF, there were comparable improvements in 6MWD (MD 37.25 metres, 95% CI 26.16 to 48.33; 278 participants; moderate-certainty evidence), peak workload (MD 9.94 watts, 95% CI 6.39 to 13.49; low-certainty evidence), VO2 (oxygen uptake) peak (MD 1.45 mL/kg/minute, 95% CI 0.51 to 2.40; low-certainty evidence) and maximum ventilation (MD 9.80 L/minute, 95% CI 6.06 to 13.53; 62 participants; low-certainty evidence). The effect of pulmonary rehabilitation on maximum heart rate was uncertain. Pulmonary rehabilitation may reduce dyspnoea in participants with ILD (standardised mean difference (SMD) -0.36, 95% CI -0.58 to -0.14; 348 participants; low-certainty evidence) and in the IPF subgroup (SMD -0.41, 95% CI -0.74 to -0.09; 155 participants; low-certainty evidence). Pulmonary rehabilitation probably improves health-related quality of life: there were improvements in all four domains of the Chronic Respiratory Disease Questionnaire (CRQ) and the St George's Respiratory Questionnaire (SGRQ) for participants with ILD and for the subgroup of people with IPF. The improvement in SGRQ Total score was -9.29 for participants with ILD (95% CI -11.06 to -7.52; 478 participants; moderate-certainty evidence) and -7.91 for participants with IPF (95% CI -10.55 to -5.26; 194 participants; moderate-certainty evidence). Five studies reported longer-term outcomes, with improvements in exercise capacity, dyspnoea and health-related quality of life still evident six to 12 months following the intervention period (6MWD: MD 32.43, 95% CI 15.58 to 49.28; 297 participants; moderate-certainty evidence; dyspnoea: MD -0.29, 95% CI -0.49 to -0.10; 335 participants; SGRQ Total score: MD -4.93, 95% CI -7.81 to -2.06; 240 participants; low-certainty evidence). In the subgroup of participants with IPF, there were improvements at six to 12 months following the intervention for dyspnoea and SGRQ Impact score. The effect of pulmonary rehabilitation on survival at long-term follow-up is uncertain. There were insufficient data to allow examination of the impact of disease severity or exercise training modality. Ten studies provided information on adverse events; however, there were no adverse events reported during rehabilitation. Four studies reported the death of one pulmonary rehabilitation participant; however, all four studies indicated this death was unrelated to the intervention received. AUTHORS' CONCLUSIONS: Pulmonary rehabilitation can be performed safely in people with ILD. Pulmonary rehabilitation probably improves functional exercise capacity, dyspnoea and quality of life in the short term, with benefits also probable in IPF. Improvements in functional exercise capacity, dyspnoea and quality of life were sustained longer term. Dyspnoea and quality of life may be sustained in people with IPF. The certainty of evidence was low to moderate, due to inadequate reporting of methods, the lack of outcome assessment blinding and heterogeneity in some results. Further well-designed randomised trials are needed to determine the optimal exercise prescription, and to investigate ways to promote longer-lasting improvements, particularly for people with IPF.

High intensity interval training versus moderate intensity continuous training for people with interstitial lung disease: protocol for a randomised controlled trial.

BACKGROUND: Interstitial lung disease is a debilitating condition associated with significant dyspnoea, fatigue, and poor exercise tolerance. Pulmonary rehabilitation is an effective and key intervention in people with interstitial lung disease. However, despite the best efforts of patients and clinicians, many of those who participate are not achieving clinically meaningful benefits. This assessor-blinded, multi-centre, randomised controlled trial aims to compare the clinical benefits of high intensity interval exercise training versus the standard pulmonary rehabilitation method of continuous training at moderate intensity in people with fibrotic interstitial lung disease. METHODS: Eligible participants will be randomised to either a standard pulmonary rehabilitation group using moderate intensity continuous exercise training or high intensity interval exercise training. Participants in both groups will undertake an 8-week pulmonary rehabilitation program of twice-weekly supervised exercise training including aerobic (cycling) and strengthening exercises. In addition, participants in both groups will be prescribed a home exercise program. Outcomes will be assessed at baseline, upon completion of the intervention and at six months following the intervention by a blinded assessor. The primary outcome is endurance time on a constant work rate test. Secondary outcomes are functional capacity (6-min walk distance), health-related quality of life (Chronic Respiratory Disease Questionnaire (CRQ), St George's Respiratory Questionnaire idiopathic pulmonary fibrosis specific version (SGRQ-I), breathlessness (Dyspnoea 12, Modified Medical Research Council Dyspnoea Scale), fatigue (fatigue severity scale), anxiety (Hospital Anxiety and Depression Scale), physical activity level (GeneActiv), skeletal muscle changes (ultrasonography) and completion and adherence to pulmonary rehabilitation. DISCUSSION: The standard exercise training strategies used in pulmonary rehabilitation may not provide an optimal exercise training stimulus for people with interstitial lung disease. This study will determine whether high intensity interval training can produce equivalent or even superior changes in exercise performance and symptoms. If high intensity interval training proves effective, it will provide an exercise training strategy that can readily be implemented into clinical practice for people with interstitial lung disease. Trial registration ClinicalTrials.gov Registry (NCT03800914). Registered 11 January 2019, https://clinicaltrials.gov/ct2/show/NCT03800914 Australian New Zealand Clinical Trials Registry ACTRN12619000019101. Registered 9 January 2019, https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=376050&isReview=true.

Home-based or remote exercise testing in chronic respiratory disease, during the COVID-19 pandemic and beyond: A rapid review.

OBJECTIVES: To identify exercise tests that are suitable for home-based or remote administration in people with chronic lung disease. METHODS: Rapid review of studies that reported home-based or remote administration of an exercise test in people with chronic lung disease, and studies reporting their clinimetric (measurement) properties. RESULTS: 84 studies were included. Tests used at home were the 6-minute walk test (6MWT, two studies), sit-to-stand tests (STS, five studies), Timed Up and Go (TUG, 4 studies) and step tests (two studies). Exercise tests administered remotely were the 6MWT (two studies) and step test (one study). Compared to centre-based testing the 6MWT distance was similar when performed outdoors but shorter when performed at home (two studies). The STS, TUG and step tests were feasible, reliable (intra-class correlation coefficients >0.80), valid (concurrent and known groups validity) and moderately responsive to pulmonary rehabilitation (medium effect sizes). These tests elicited less desaturation than the 6MWT, and validated methods to prescribe exercise were not reported. DISCUSSION: The STS, step and TUG tests can be performed at home, but do not accurately document desaturation with walking or allow exercise prescription. Patients at risk of desaturation should be prioritised for centre-based exercise testing when this is available.

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.

Greater endurance capacity and improved dyspnoea with acute oxygen supplementation in idiopathic pulmonary fibrosis patients without resting hypoxaemia.

BACKGROUND AND OBJECTIVE: Supplemental oxygen is commonly prescribed in patients with idiopathic pulmonary fibrosis (IPF), although its benefits have not been proven. The aims of this study were to investigate the effect of oxygen on oxidative stress, cytokine production, skeletal muscle metabolism and physiological response to exercise in IPF. METHODS: Eleven participants with IPF received either oxygen, at an FiO2 of 0.50, or compressed air for 1 h at rest and during a cycle endurance test at 85% of peak work rate. Blood samples collected at rest and during exercise were analysed for markers of oxidative stress, skeletal muscle metabolism and cytokines. The protocol was repeated a week later with the alternate intervention. RESULTS: Compared with air, oxygen did not adversely affect biomarker concentrations at rest and significantly improved endurance time (mean difference = 99 ± 81s, P = 0.002), dyspnoea (-1 ± 1 U, P = 0.02), systolic blood pressure (BP; -11 ± 11 mm Hg, P = 0.006), nadir oxyhaemoglobin saturation (SpO2 ; 8 ± 6%, P = 0.001), SpO2 at 2-min (7 ± 6%, P = 0.003) and 5-min isotimes (5 ± 3, P < 0.001) and peak exercise xanthine concentrations (-42 ± 73 µmol/L, P = 0.03). Air significantly increased IL-10 (5 ± 5 pg/mL, P = 0.04) at 2-min isotime. Thiobarbituric acid-reactive substances (TBARs), IL-6, TNF-α, creatine kinase, lactate, heart rate and fatigue did not differ between the two interventions at any time point. CONCLUSION: In patients with IPF, breathing oxygen at FiO2 of 0.50 at rest seems safe. During exercise, oxygen improves exercise tolerance, alleviates exercise-induced hypoxaemia and reduces dyspnoea. A potential relationship between oxygen administration and improved skeletal muscle metabolism should be explored in future studies.

Australian and New Zealand Pulmonary Rehabilitation Guidelines.

BACKGROUND AND OBJECTIVE: The aim of the Pulmonary Rehabilitation Guidelines (Guidelines) is to provide evidence-based recommendations for the practice of pulmonary rehabilitation (PR) specific to Australian and New Zealand healthcare contexts. METHODS: The Guideline methodology adhered to the Appraisal of Guidelines for Research and Evaluation (AGREE) II criteria. Nine key questions were constructed in accordance with the PICO (Population, Intervention, Comparator, Outcome) format and reviewed by a COPD consumer group for appropriateness. Systematic reviews were undertaken for each question and recommendations made with the strength of each recommendation based on the GRADE (Gradings of Recommendations, Assessment, Development and Evaluation) criteria. The Guidelines were externally reviewed by a panel of experts. RESULTS: The Guideline panel recommended that patients with mild-to-severe COPD should undergo PR to improve quality of life and exercise capacity and to reduce hospital admissions; that PR could be offered in hospital gyms, community centres or at home and could be provided irrespective of the availability of a structured education programme; that PR should be offered to patients with bronchiectasis, interstitial lung disease and pulmonary hypertension, with the latter in specialized centres. The Guideline panel was unable to make recommendations relating to PR programme length beyond 8 weeks, the optimal model for maintenance after PR, or the use of supplemental oxygen during exercise training. The strength of each recommendation and the quality of the evidence are presented in the summary. CONCLUSION: The Australian and New Zealand Pulmonary Rehabilitation Guidelines present an evaluation of the evidence for nine PICO questions, with recommendations to provide guidance for clinicians and policymakers.

Principles of rehabilitation and reactivation: interstitial lung disease, sarcoidosis and rheumatoid disease with respiratory involvement.

The interstitial lung diseases (ILDs) are characterised by dyspnoea on exertion, exercise-induced hypoxaemia, reduced skeletal muscle function and exercise intolerance. Evidence from nine randomised controlled trials shows that pulmonary rehabilitation improves exercise capacity, dyspnoea and quality of life in ILD, with moderately large effect sizes from 0.59 to 0.68. Participants with idiopathic pulmonary fibrosis, the most common and most progressive of the ILDs, achieve benefits in exercise capacity and quality of life that are of equal magnitude to those seen in other ILDs, with effect sizes from 0.59 to 0.75. Whole body exercise training is a core component of pulmonary rehabilitation for ILD. The standard exercise prescription used for other chronic lung diseases is effective in ILD, including 8 weeks of training with at least two supervised sessions per week and at least 30 min of aerobic training per session. However, the unique presentation and underlying pathophysiology of ILD may require modifications of the exercise prescription for individual patients. Those with connective tissue disease may present with joint pain and stiffness that require modification of the standard exercise prescription, including reduction in weight-bearing exercise. Some patients with severe disease may present with distressing dyspnoea that limits the intensity or progression of training. Because exercise-induced hypoxaemia is common in ILD and more severe than seen in other chronic lung diseases, pulmonary rehabilitation should be provided in a setting where supplemental oxygen therapy is available. Pulmonary rehabilitation programs offer the opportunity to address other critical aspects of ILD care, including management of comorbidities, symptoms and mood.

Be honest and help me prepare for the future: What people with interstitial lung disease want from education in pulmonary rehabilitation.

Pulmonary rehabilitation (PR) is recommended for people with interstitial lung disease (ILD); however, the educational content of PR was not designed for this group. This study explored the perspectives of patients and ILD clinicians regarding the educational content of PR for ILD. A qualitative study using individual semi-structured interviews was undertaken. Transcripts were coded independently by two investigators and themes established by consensus. Participants were 18 people with ILD (9 idiopathic pulmonary fibrosis, diffusing capacity for carbon monoxide 54 (20)% predicted) and 14 clinicians from 5 countries and 5 disciplines. Major themes from patient interviews were the importance of knowing what the future might bring and the need for honesty from clinicians. Most were happy to attend standard PR education sessions but wanted ILD-specific content. Patients wanted information about end-of-life planning and most were happy to discuss it in a group. Among clinicians, there was no consensus regarding whether prognosis should be discussed in PR. Most clinicians supported discussion of advanced care planning, however, some thought it should not be discussed in a group. We conclude that people with ILD have specific educational needs that may not be met in the current PR format. Patients and clinicians have some discordant views about programme content.

Dyspnoea and comorbidity contribute to anxiety and depression in interstitial lung disease.

BACKGROUND AND OBJECTIVES: Little is known about the prevalence of anxiety in interstitial lung disease (ILD), and the contributors to depression are not clear. The aim of this study was to determine the prevalence and predictors of anxiety and depression in people with ILD. METHODS: One hundred and twenty-four individuals with ILD (age 64 years (standard deviation 12), 48 idiopathic pulmonary fibrosis) participated. Anxiety and depression were assessed using the Hospital Anxiety and Depression Scale to determine likely cases and borderline cases. Associations with demographic data, respiratory function, 6-min walk and Modified Medical Research Council Dyspnoea Scale (MMRC) were examined. RESULTS: The prevalence of anxiety was 31%, with clinically significant anxiety in 12%. Depression was present in 23% of individuals, with 7% having clinically significant depression. Independent predictors of anxiety were a higher MMRC score (P = 0.005, odds ratio (OR) for case 2.60, 95% confidence interval 1.37 to 4.92) and higher nadir SpO2 during walking (P = 0.003, OR for case 1.16, 1.04-1.30). Independent predictors of depression were a higher MMRC score (P = 0.006, case OR 3.84, 1.25-11.78, borderline case OR 2.44, 1.14-5.19) and a greater number of comorbidities (P = 0.003, case OR 2.02, 0.97-4.21, borderline case OR 2.26, 1.30-3.93). CONCLUSIONS: Anxiety and depression are present in a significant minority of individuals with ILD. Dyspnoea and comorbidities are important contributors that may be amenable to intervention.

Pulmonary rehabilitation for interstitial lung disease.

BACKGROUND: Interstitial lung disease (ILD) is characterised by reduced functional capacity, dyspnoea and exercise-induced hypoxia. Pulmonary rehabilitation, an intervention that includes exercise training, is beneficial for people with other chronic lung conditions; however its effects in ILD have not been well characterised. OBJECTIVES: • To determine whether pulmonary rehabilitation in patients with ILD has beneficial effects on exercise capacity, symptoms, quality of life and survival compared with no pulmonary rehabilitation in patients with ILD.• To assess the safety of pulmonary rehabilitation in patients with ILD. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL) (2014, Issue 6), MEDLINE (Ovid), EMBASE (Ovid), the Cumulative Index to Nursing and Allied Health Literature (CINAHL) (EBSCO) and the Physiotherapy Evidence Database (PEDro) (all searched from inception to June 2014). We also searched the reference lists of relevant studies, international clinical trial registries and respiratory conference abstracts to look for qualifying studies. SELECTION CRITERIA: Randomised and quasi-randomised controlled trials in which pulmonary rehabilitation was compared with no pulmonary rehabilitation or with other therapy in people with ILD of any origin were included. DATA COLLECTION AND ANALYSIS: Two review authors independently selected trials for inclusion, extracted data and assessed risk of bias. Study authors were contacted to provide missing data and information regarding adverse effects. A priori subgroup analyses were specified for participants with idiopathic pulmonary fibrosis (IPF) and participants with severe lung disease (low diffusing capacity or desaturation during exercise). We planned to subgroup according to training modality applied, but there were insufficient data. MAIN RESULTS: Nine studies were included, six of which were published as abstracts. Five studies were included in the meta-analysis (86 participants who undertook pulmonary rehabilitation and 82 control participants). One study used a blinded assessor and intention-to-treat analysis. No adverse effects of pulmonary rehabilitation were reported. Pulmonary rehabilitation improved the six-minute walk distance with weighted mean difference (WMD) of 44.34 metres (95% confidence interval (CI) 26.04 to 62.64 metres) and improved oxygen consumption (VO2) peak with WMD of 1.24 mL/kg/min(-1) (95% CI 0.46 to 2.03 mL/kg/min(-1)). Improvements in six-minute walk distance and VO2 peak were also seen in the subgroup of participants with idiopathic pulmonary fibrosis (IPF) (WMD 35.63 metres, 95% CI 16.02 to 55.23 metres; WMD 1.46 mL/kg/min(-1), 95% CI 0.54 to 2.39 mL/kg/min(-1), respectively). Reduced dyspnoea (standardised mean difference (SMD) -0.66, 95% CI -1.05 to -0.28) following pulmonary rehabilitation was also seen in the IPF subgroup (SMD -0.68, 95% CI -1.12 to -0.25). Quality of life improved following pulmonary rehabilitation for all participants on a variety of measures (SMD 0.59, 95% CI 0.20 to 0.98) and for the subgroup of people with IPF (SMD 0.59, 95% CI 0.14 to 1.03). Two studies reported longer-term outcomes, with no significant effects of pulmonary rehabilitation on clinical variables or survival at three or six months. Available data were insufficient to allow examination of the impact of disease severity or exercise training modality. AUTHORS' CONCLUSIONS: Pulmonary rehabilitation seems to be safe for people with ILD. Improvements in functional exercise capacity, dyspnoea and quality of life are seen immediately following pulmonary rehabilitation, with benefits also evident in IPF. Because of inadequate reporting of methods and small numbers of included participants, the quality of evidence was low to moderate. Little evidence was available regarding longer-term effects of pulmonary rehabilitation.

Cardiorespiratory responses to 6-minute walk test in interstitial lung disease: not always a submaximal test.

BACKGROUND: The 6-minute walk test (6 MWT) is used to measure exercise capacity and assess prognosis in interstitial lung disease (ILD). Although the 6 MWT is usually considered to be a test of submaximal exercise capacity in ILD, the physiological load imposed by this test is not well described and 6 MWT outcomes are poorly understood. This study aimed to compare cardiorespiratory responses to 6 MWT and cardiopulmonary exercise test (CPET) in people with ILD. METHODS: 47 participants with ILD (27 idiopathic pulmonary fibrosis (IPF), mean age 71 (SD 12) years, diffusing capacity for carbon monoxide (TLCO) 49(15) %predicted) undertook CPET and 6 MWT on the same day in random order. Oxygen uptake (VO(2)), ventilation (VE) and carbon dioxide production (VCO2) were assessed during each test using a portable metabolic cart. RESULTS: The VO(2)peak during the 6 MWT was lower than during CPET (1.17(0.27) vs 1.30(0.37) L.min-1, p = 0.001), representing an average of 94% (range 62-135%) of CPET VO(2)peak. Achieving a higher percentage of CPET VO(2)peak on 6 MWT was associated with lower TLCO %predicted (r = -0.43, p = 0.003) and more desaturation during walking (r = -0.46, p = 0.01). The VEpeak and VCO(2)peak were significantly lower during 6 MWT than CPET (p < 0.05). However, participants desaturated more during the 6 MWT (86(6)% vs 89(4)%, p < 0.001). The degree of desaturation was not affected by the percent of peak VO2 achieved during the 6 MWT. Responses were similar in the subgroup with IPF. CONCLUSIONS: On average, the 6 MWT elicits a high but submaximal oxygen uptake in people with ILD. However the physiological load varies between individuals, with higher peak VO2 in those with more severe disease that may match or exceed that achieved on CPET. The 6 MWT is not always a test of submaximal exercise capacity in people with ILD.

The benefits of exercise training in interstitial lung disease: protocol for a multicentre randomised controlled trial.

BACKGROUND: Interstitial lung disease encompasses a diverse group of chronic lung conditions characterised by distressing dyspnoea, fatigue, reduced exercise tolerance and poor health-related quality of life. Exercise training is one of the few treatments to induce positive changes in exercise tolerance and symptoms, however there is marked variability in response. The aetiology and severity of interstitial lung disease may influence the response to treatment. The aims of this project are to establish the impact of exercise training across the range of disease severity and to identify whether there is an optimal time for patients with interstitial lung disease to receive exercise training. METHODS/DESIGN: One hundred and sixteen participants with interstitial lung disease recruited from three tertiary institutions will be randomised to either an exercise training group (supervised exercise training twice weekly for eight weeks) or a usual care group (weekly telephone support). The 6-minute walk distance, peripheral muscle strength, health-related quality of life, dyspnoea, anxiety and depression will be measured by a blinded assessor at baseline, immediately following the intervention and at six months following the intervention. The primary outcome will be change in 6-minute walk distance following the intervention, with planned subgroup analyses for participants with idiopathic pulmonary fibrosis, dust-related interstitial lung disease and connective-tissue related interstitial lung disease. The effects of disease severity on outcomes will be evaluated using important markers of disease severity and survival, such as forced vital capacity, carbon monoxide transfer factor and pulmonary hypertension. DISCUSSION: This trial will provide certainty regarding the role of exercise training in interstitial lung disease and will identify at what time point within the disease process this treatment is most effective. The results from this study will inform and optimise the clinical management of people with interstitial lung disease. TRIAL REGISTRATION: Australian New Zealand Clinical Trials Registry ACTRN12611000416998.

Prescribing walking training in interstitial lung disease from the 6-minute walk test.

INTRODUCTION AND OBJECTIVE: Endurance training during PR requires exercise prescription at sufficient intensity to achieve physiological benefits. This analysis sought to investigate whether walking training prescribed from 6-minute walk test (6MWT) average speed provides an appropriate training intensity for people with ILD during PR. METHODS: Individuals with ILD completed cardiopulmonary exercise test (CPET) and 6MWT in random order. A 10-minute constant speed treadmill walk test (10MTW) was undertaken at 80% of the average 6MWT speed. Oxygen uptake (VO2) was measured during all tests. Percentage VO2peak during 10MTW was main outcome measure. RESULTS: Eleven people with ILD (age 71 (8) years; forced vital capacity 73 (18) %predicted, 6-minute walk distance 481 (99) meters, and VO2peak during CPET 1.3 (0.2) L.min-1) undertook testing. Average VO2peak during 10MTW was 91 (18) % of CPET VO2peak [range 67-116%]. Participants who achieved a greater VO2peak during CPET walked at a smaller %VO2peak during 10MTW (r = -0.6; p = .04). CONCLUSIONS: For people with ILD, walking training prescribed at 80% of 6MWT average speed can provide adequate exercise training intensity for PR.

Self-management interventions for people with pulmonary fibrosis: a scoping review.

BACKGROUND: The most effective method for encouraging self-management in individuals with pulmonary fibrosis (PF) is unclear. This review aimed to identify common self-management components, the outcome measures used and the impact of these components in PF. METHODS: A scoping review was conducted according to the Joanna Briggs Institute Manual for Evidence Synthesis using Medline, Embase, PsychInfo, CINAHL and the Cochrane Central Register of Controlled Trials. Eligible studies included those with educational, behavioural or support components aimed at facilitating self-management among adults with PF and employed quantitative and/or qualitative methods. RESULTS: 87 studies were included. Common self-management components included education (78%), managing physical symptoms (66%) and enhancing psychosocial wellbeing (54%). Components were predominantly delivered in a pulmonary rehabilitation setting (71%). No studies tested a PF-specific self-management package. Common outcome measures were 6-min walk distance (60%), St George's Respiratory Questionnaire (37%) and the Medical Research Council Dyspnoea scale (34%). Clinically significant improvements in these outcomes were seen in ≥50% of randomised controlled trials. Qualitative data highlighted the importance of healthcare professional and peer support and increased confidence in managing PF. CONCLUSION: Self-management components are commonly incorporated into pulmonary rehabilitation programmes rather than being offered as standalone packages. Future research should focus on testing PF-specific self-management packages and employ standardised outcome assessments that include self-efficacy and health-related behaviours.