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.

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.

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.

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.

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.

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.

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.

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.

Short-Term Improvement in Physical Activity and Body Composition After Supervised Exercise Training Program in Idiopathic Pulmonary Fibrosis.

OBJECTIVE: To examine the effect of participating in a 12-week supervised exercise training (ET) program on physical activity and body composition in patients with idiopathic pulmonary fibrosis (IPF). DESIGN: Randomized controlled trial assessing physical activity and body composition at baseline, after 12-week intervention, and at 11 months follow-up. SETTING: Outpatient hospital. PARTICIPANTS: Patients with IPF (N=34; mean age, 68±8y) recruited for this study; 32 patients completed the 12-week intervention (ET group: n=15; control group: n=17) and 28 patients (14 in each group) reassessed at 11-month follow-up from baseline. INTERVENTIONS: Participation in a 12-week supervised ET program or regular medical treatment. MAIN OUTCOME MEASURES: Changes in physical activity levels as measured by the International Physical Activity Questionnaire. RESULTS: After the 12-week intervention, physical activity levels were significantly increased in the ET group whereas the control group showed a trend toward deterioration (median difference between the groups, 2164MET-min/wk [interquartile range, 1576MET-min/wk]; MET = metabolic equivalent; P<.001). Body composition was also significantly improved in the ET group, whereas the control group showed an opposite trend. At 11-month follow-up, no significant differences were observed between the 2 groups in all variables. The ET group lost most of the achieved improvements in the outcomes and returned to near baseline levels, whereas the control group showed a trend toward worsened outcomes. CONCLUSIONS: Physical activity and body composition in patients with IPF were improved after a 12-week supervised ET program, although the benefits were not sustained at 11-month follow-up. These results may support the efficacy of participation in supervised ET to improve physical activity and body composition in patients with IPF; however, maintenance strategies are warranted to preserve the improved outcomes.

[Perioperative Pulmonary Rehabilitation for Lung Cancer Surgeries in Patients with Poor Pulmonary Function].

To properly perform preoperative pulmonary rehabilitation is important for lung cancer surgeries in patients with poor pulmonary function such as severe chronic obstructive pulmonary disease( COPD) to prevent postoperative complications. Those programs include exercise training, pursed-lip breathing technique, activities of dairy living training and facilitating physical activities, all which are almost same as those for patients with stable COPD. Pedometer is a useful tool to lead patient's physical activities. Postoperative therapeutic programs are also important, which includes early mobilization, nutritional support, and so on.

Evaluation of activity limitation in patients with idiopathic pulmonary fibrosis grouped according to Medical Research Council dyspnea grade.

OBJECTIVE: To investigate the relations between Medical Research Council (MRC) dyspnea grade and peripheral muscle force, activities of daily living (ADL) performance, health status, lung function, and exercise capacity in subjects with idiopathic pulmonary fibrosis (IPF). DESIGN: Prospective cross-sectional observational study. SETTING: University hospital. PARTICIPANTS: Subjects with IPF (N=65, 46 men) in a stable clinical state with a mean age of 68±7 years. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Right ventricular systolic pressure (RVSP) via transthoracic echocardiography, pulmonary function, isometric quadriceps force (QF) and handgrip force (HF), 6-minute walk distance (6MWD), ADL score, and health status (Medical Outcomes Study 36-Item Short-Form Health Survey [SF-36]) were assessed and compared between subjects grouped according to MRC grade. RESULTS: Of the participants, 16 were in MRC grade 2, 17 were in MRC grade 3, 17 were in MRC grade 4, and 15 were in MRC grade 5. RVSP, pulmonary function, QF, HF, 6MWD, ADL, and SF-36 scores decreased with increasing MRC grade (all P<.001). All measures were lower (P<.05) in subjects with grades 4 and 5 than subjects with grades 2 and 3. Strong associations were found between MRC grade and 6MWD (ρ=-.89, P=.001) and ADL score (ρ=-.82, P=.001). MRC grade was also associated with RVSP, pulmonary function, QF, and HF (all ρ≥.56, P=.001). CONCLUSIONS: The MRC dyspnea scale provides a simple and useful method of categorizing individuals with IPF with respect to their activity limitation and may assist in understanding the impact of IPF on an individual.

Exercise training-based pulmonary rehabilitation program is clinically beneficial for idiopathic pulmonary fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic, devastating, interstitial lung disease, with few therapeutic options. IPF is characterized by pulmonary restriction, dyspnea, hypoxemia, exercise intolerance and poor quality of life (QOL). OBJECTIVES: We aimed to examine the effect of exercise training (ET) on clinical outcomes in IPF patients. METHODS: A randomized controlled study included thirty-two IPF patients (aged 68 ± 8 years) who were allocated either to the ET group (n = 15), participating in a 12-week, twice-weekly 60-min supervised ET-based pulmonary rehabilitation program, or to a control group (n = 17) continuing with regular medical treatment alone. Cardiopulmonary exercise test, 6-min walking distance (6MWD) test, 30-second chair-stand test, pulmonary function tests, dyspnea and QOL were assessed at baseline and at the end of the 12-week intervention. RESULTS: Significant differences were observed between the ET and the control groups in raw mean deltas (Δ = post- - pre-intervention): Δ6MWD, 81 m, p < 0.001; ΔVO2 peak, 2.6 ml/kg/min, p = 0.002; Δwork rate, 22 W, p < 0.001; Δanaerobic threshold, 3.1 ml/kg/min, p < 0.001, and ΔFVC % predicted, 6%, p = 0.038. Dyspnea, QOL and 30-second chair-stand were also improved significantly following the program. CONCLUSIONS: ET improves exercise tolerance, functional capacity, pulmonary function, dyspnea and QOL in patients with IPF, suggesting a short-term treatment efficacy for clinical improvement, and should be considered the standard care for IPF.

Exercise limitation in IPF patients: a randomized trial of pulmonary rehabilitation.

BACKGROUND: Patients with idiopathic pulmonary fibrosis (IPF) have severely limited exercise capacity due to dyspnea, hypoxemia, and abnormal lung mechanics. This pilot study was designed to determine whether pulmonary rehabilitation were efficacious in improving the 6-min walk test (6-MWT) distance, exercise oxygen uptake, respiratory muscle strength [maximum inspiratory pressure (MIP)], and dyspnea in patients with IPF. Underlying physiological mechanisms and effects of the intervention were investigated. METHODS: Subjects were randomly assigned to a 3-month pulmonary rehabilitation program (n = 11) or to a control group (n = 10). All subjects initially underwent the 6-MWT and constant load exercise gas exchange studies. RESULTS: Subjects in the rehabilitation group increased treadmill exercise [metabolic equivalent of task-minutes] over the first 14 sessions. Beneficial effects on physical function resulted in those who completed rehabilitation. Subjects who completed the program increased cycle ergometer time and maintained exercise oxygen consumption (exercise VO(2)) at the baseline level over 3 months, while the control group suffered a significant decrease in exercise VO(2). Rehabilitation subjects also increased their MIP. Plasma lactate doubled and brain natriuretic peptide levels increased significantly after exercise, as did the plasma amino acids glutamic acid, arginine, histidine, and methionine. These changes were associated with significant decreases in arterial oxygen saturation and increases in 15-F(2t)-isoprostanes after exercise. CONCLUSIONS: Pulmonary rehabilitation effectively maintained exercise oxygen uptake over 3 months and lengthened constant load exercise time in patients with moderately severe IPF. Exercise endurance on cycle ergometry testing was limited by dyspnea and severe hypoxemia associated with systemic oxidant stress.

Referral to pulmonary rehabilitation and palliative care services in people with idiopathic pulmonary fibrosis in England, 2010-2019.

The benefits of pulmonary rehabilitation (PR) and palliative care (PC) as non-pharmacological therapies for people with idiopathic pulmonary fibrosis (IPF) are increasingly being recognised but in the UK the proportion of people with this life-limiting condition who are referred to such services is thought to be low. This retrospective cohort study aimed to describe trends in referrals to PR and PC services among people with IPF over a 10-year period and to identify factors associated with non-referral. Our study cohort was drawn from the UK's pseudonymised Clinical Practice Research Datalink (CPRD) Aurum primary care database and comprised 17,071 individuals diagnosed with IPF between 2010 and 2019. While 12.0% of IPF patients were offered a referral to PR, less than 2% completed a PR programme. Around a fifth (19.4%) received a referral to generic PC support services; however, this is well below reported PC referral rates for lung cancer patients. Moreover, the majority of PC referrals occurred late; among those who died, 31% were referred within a month and 70% within 6 months of death. Referrals to PR and PC had however increased (by around 2-fold and 4-fold, respectively) over the course of the study period. Factors associated with non-referral to PR included female sex, older age and co-diagnosis of dementia; barriers to PC referral included being female or of Asian or Black ethnicity. We also found evidence of regional differences in referrals. These findings confirm that PR and PC service provision for people with IPF across England is suboptimal.

Role of support measures and palliative care.

PURPOSE OF REVIEW: The care of patients with idiopathic pulmonary fibrosis is challenging at a time when there are no medications with proven efficacy in extending patient survival or quality of life. However, a number of recent studies suggest that patients participating in clinical trials including the placebo arm have improved outcome. This suggests that there are elements of supportive care which may prove beneficial to IPF patients. Further, control of patient symptoms should be a critical goal in care of patients with IPF. RECENT FINDINGS: A number of both pharmacologic and nonpharmacologic therapies are available to reduce symptoms in patients with IPF. These include low-dose narcotics, pulmonary rehabilitation and oxygen therapy. Further, addressing issues of depression may be beneficial for symptom management. Palliative care can and should be more fully integrated in the care of patients with IPF. SUMMARY: Incorporating supportive and palliative measures in the care of patients with IPF may improve both quality of life and survival, but far more research is needed in this fledgling field.

Pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis--a review.

Among the various types of interstitial lung diseases, idiopathic pulmonary fibrosis (IPF) is the most common disorder and has a poor prognosis and a limited response to pharmacological treatment. In patients with IPF, functional exercise tolerance and quality of life have been shown to be significantly decreased. Current IPF guidelines suggest only a weak recommendation for pulmonary rehabilitation (PR). However, PR is regarded as a reasonable choice for the majority of patients with IPF. This review will summarize all of the available studies that have investigated the effects of PR in patients with IPF so far. Although only a small number of studies have been published to date, most studies have found significant short-term improvements in functional exercise capacity, quality of life, and level of perceived dyspnea. Long-term improvements or maintenance strategies of PR in IPF patients have not been adequately investigated yet. Up to now there is still no sufficient evidence for the recommendation of PR in IPF. However, physical training seems to be the major component of all PR programs. The current review will discuss potential exercise training regimens for patients with IPF and suggest additional useful modalities of a specific multidisciplinary PR program for IPF patients. Based on the current literature and our own experience, this article will try to highlight the importance of PR as an additional, beneficial therapeutic option for patients with IPF.

Depression in patients with idiopathic pulmonary fibrosis.

Depression carries enormous global morbidity and is 1.5-7 times likelier to occur in individuals with chronic illness than in the general population. Idiopathic pulmonary fibrosis (IPF) has a rising incidence with a severe impact on quality of life. An indication of the prevalence of depression in this group is therefore of paramount interest. A prospective study was performed. A total of 118 participants with IPF who attended the interstitial lung disease clinic in Ninewells Hospitals, Dundee, Scotland, from May 2010 to September 2011 were recruited. Informed consent was obtained. The male to female ratio was 60:58. The Wakefield Self-assessment of Depression Inventory was used (scores ≥15 denote a depressed state). Pulmonary function tests were measured to correlate disease severity with depression scores. Of them, 58 patients had significant depressive symptoms scoring ≥15; only nine were taking antidepressant medication. The mean depression score of female participants was 15.0 ± 0.77 (SD 5.9), compared with a mean male score of 13.1 ± 0.99 (SD 7.5). Disease severity, age, duration since diagnosis and number of co-morbidities were not significantly correlated with depression. The study population had a high prevalence of depressive symptoms. Medical therapy for pulmonary fibrosis is limited and therefore palliation of symptoms and pulmonary rehabilitation form the main strategy for management. Depression should be actively screened in patients with IPF.

Idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis is a progressive lung disease that carries a poor prognosis and for which there are no effective therapies. Although the excessive deposition of extracellular matrix, combined with evidence of recurrent injury to the alveolar epithelium, are well-described there is a pressing need to understand these processes better at a molecular level and thus to identify potential therapeutic targets in this intractable disease. This review considers some recent advances published in Thorax and elsewhere that have improved our understanding of the pathophysiology of idiopathic pulmonary fibrosis, using data both from human cells and tissue and from animal models of pulmonary fibrosis. The studies particularly address the fate of the alveolar epithelial cell and mechanisms of fibrogenesis, and identify mechanistic pathways shared with co-existing conditions such as lung cancer and pulmonary hypertension. The concepts of physiological biomarkers of disease progression and prognosis are also discussed.

[The role of pulmonary rehabilitation in idiopathic pulmonary fibrosis].

Idiopathic pulmonary fibrosis (IPF) is the most common interstitial lung disease (ILD) characterized by exertional dyspnea, hypoxemia and exercise intolerance. The pathophysiology of IPF usually presents restrictive physiology of lung volume and impaired gas exchange. Most of the research which supports the effectiveness of pulmonary rehabilitation (PR) comes from chronic obstructive pulmonary disease (COPD) patients' studies. However, in the past few years growing evidence has emerged to support the effectiveness and safety of PR programs in IPF patients. Exercise training in PR programs was shown to increase exercise and functional capacity, decreasing shortness of breath and improving quality of life in IPF patients. Nevertheless, PR guidelines for IPF patients have not yet been established. This review presents the current existing data on the effectiveness of PR programs in IPF patients. Large and well controlled trials are needed for the establishment of clinical guidelines of PR in IPF patients.

Effect of disability level on response to pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis.

BACKGROUND AND OBJECTIVE: It is unclear whether the severity of functional limitation resulting from IPF affects the response to pulmonary rehabilitation. The aim of this study was to compare the outcomes of rehabilitation in patients with IPF, who were grouped according to the Medical Research Council (MRC) dyspnoea scale. METHODS: Sixty-five subjects (46, 71% men) with stable IPF were enrolled in an 8-week pulmonary rehabilitation programme. Subjects with MRC dyspnoea grades 2, 3 and 4 undertook a supervised outpatient programme, whereas subjects with MRC dyspnoea grade 5 participated in an unsupervised, home-based programme, with review every 2 weeks. The outcome measures included functional exercise capacity (6MWD), health status (Medical Outcomes Study Short Form 36 (SF-36)) and dyspnoea (transition dyspnoea index), which were measured at baseline and immediately after the programme. Hospitalizations for respiratory exacerbations were compared for the 12 months preceding and following the programme. RESULTS: The number of subjects with MRC dyspnoea grades 2, 3, 4 and 5 were 16 (25%), 17 (26%), 17 (26%) and 15 (23%), respectively. There were differences between these groups in the magnitude of change in 6MWD, SF-36 and transition dyspnoea index (all P < 0.05). Specifically, subjects with MRC dyspnoea grade 2 or 3 demonstrated clinically and statistically significant improvements in 6MWD and SF-36 following rehabilitation (all P < 0.05). In contrast, for all measures, subjects with MRC dyspnoea grade 4 or 5 showed little or no improvement, or deteriorated following rehabilitation. Hospitalizations were reduced following rehabilitation only in subjects with MRC dyspnoea grade 2, 3 or 4 (P<0.05). CONCLUSIONS: The response to pulmonary rehabilitation in subjects with IPF varies depending on the MRC grade of dyspnoea, with little benefit being observed in subjects with severe functional limitation.