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Abstract

Background

Individuals with interstitial lung disease (ILD) often experience worsening symptoms and activity avoidance. Limited data exists on outcome measures for assessing functional status (capacity and performance), as well as on the effectiveness of pulmonary rehabilitation (PR) in improving these outcomes in ILD.

Aim

This review aimed to systematically assess the effects of PR on both functional capacity and performance in individuals with ILD.

Methods

Randomised controlled trials involving pulmonary rehabilitation (PR) in adults with ILD, which included at least an exercise training component and education and/or psychosocial support, were included. Risk of bias and quality of evidence were assessed. Mean changes from baseline and standard deviations were retrieved for each group, and a random-effects model was applied.

Results

Eight studies were included, mostly involving individuals with idiopathic pulmonary fibrosis (n = 5). PR duration ranged from 3 to 26 weeks. Seven studies used the 6MWT to evaluate functional capacity and one also used the 30-s STS. Two studies assessed functional performance, measuring time spent in moderate physical activity with the SenseWear Armband, number of steps per day with the same device, and energy expenditure in MET-min using the international physical activity questionnaire. PR improved functional capacity (6MWT-MD 45.82 m, 95%CI [26.14; 65.50], I² = 71.54%, p < .001; 30-s STS- PR: 3.7 ± 2.6 reps; control group: −0.4 ± 2.5 reps, p < .001) compared to usual care. Only self-reported physical activity levels increased after PR (PR: 51.4 ± 57.7MET-min; control group: 20.9 ± 37.2MET-min, p = .03).

Conclusion

PR is effective at improving functional capacity; however, functional performance is often overlooked, resulting in limited and inconclusive findings.

Keywords

“Interstitial lung diseases”Rehabilitation “Functional capacity”“Functional performance”

Introduction

Individuals with interstitial lung disease (ILD) often experience worsening symptoms, leading to limitations in their ability to perform activities¹ and participate in daily life, thus affecting their functional status. Functional status, recently identified as a research priority in ILD,² encompasses two main yet complementary concepts: functional capacity and functional performance.³ Functional capacity refers to a person’s maximum potential to perform a functional activity (e.g., walking, sitting, standing, or stair climbing) under controlled, standardised conditions, and it is typically measured through functional tests.³ Functional performance refers to what people actually do in the context of their daily lives and is mostly assessed through patient-reported outcome measures (PROMs), which may evaluate whether patients can perform activities of daily living or assess the impact of specific barriers to activity (such as dyspnoea).⁴ Quality of life measures, which can include sub-domains related to activities, may also be used; however, the data they provide serve as proxies for functional performance.⁴ Physical activity (PA) is also considered functional performance because it involves the volume and intensity of activities people engage in during their daily lives.³ Its assessment may be conducted with PROMs or objective measurements, such as activity monitors.⁵ Functional status is, therefore, a complex construct that is highly meaningful to patients and can potentially be improved through non-pharmacological interventions. Pulmonary rehabilitation (PR) is a non-pharmacological intervention that has been found to be safe and effective for individuals with ILD^6,8; however, adherence to PR standards, which require exercise training and educational/psychosocial support as minimum components,⁷ varies among studies. Published systematic reviews^6,8 have provided evidence from studies that adhere to the definition of PR; however, they also include results from exercise-only interventions. Combining data from these studies without recognising key differences hampers our understanding of the true impact of PR in several domains, including functional status in ILD. Moreover, there is limited data on the outcome measures used to assess functional capacity and performance in ILD, as no review has focused on summarizing these health domains. Hence, this review aimed to systematically assess the effects of PR on both functional capacity and performance in adults with ILD.

Methods

This systematic literature review was reported according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement⁹ (Section A1). The review protocol was registered at the International Prospective Register of Systematic Reviews (CRD42022298584).

Eligibility criteria

Studies were included if they: i) were randomised controlled trials (RCTs); ii) included individuals aged ≥18 years with a clinical diagnosis of ILD; iii) implemented PR programmes including, at least, an exercise training component (e.g., strength and/or endurance training) and education and/or psychosocial support; iv) compared PR to usual care or any other intervention; v) reported outcome measures of functional capacity and/or performance and vi) were written in Portuguese, English, French or Spanish. Although not explicitly outlined in our registration protocol, RCTs with alternative models of PR delivery (e.g., home-based, telerehabilitation) were included if they involved supervised sessions; otherwise, they were excluded. Qualitative studies, grey/unpublished literature and studies involving mixed chronic respiratory diseases or non-traditional exercise modalities (e.g., yoga, tai chi, qigong) were excluded.

Search strategy

The search was first conducted in December 2022 and rerun in March 2024 in PubMed/MEDLINE, Scopus, and Web of Science Core Collection. The complete search strategies for all databases can be found in the supplementary material (Section A1). CINAHL and EMBASE were not searched as originally planned because our institution’s subscription was no longer available. The reference lists of the articles included in the review, as well as those of published systematic reviews^5,6,8 were examined to identify additional research studies. Citations were imported into EndNote 20®. After duplicates were removed, all references were screened by two independent reviewers based on the title and abstract. Articles that met the inclusion criteria, or were unclear regarding inclusion, were screened out, and the full text of each article was carefully reviewed before making a final decision. Another reviewer was consulted to clarify any discrepancies. Interrater agreement was measured using Cohen’s Kappa statistic (0.00-0.20 slight; 0.21-0.40 fair; 0.41-0.60 moderate; 0.61-0.80 substantial; 0.81-1.00 perfect agreement).¹⁰

Data extraction and analysis

A reviewer collected information from included studies into a predefined Microsoft Excel spreadsheet, allowing for easy comparisons and reliable synthesis of results. The extracted data included patient characteristics (age, sex, lung function), details of the intervention, and means and standard deviations (SD) of baseline and follow-up measurements for each outcome of interest. In case of missing summary statistics, reviewers: i) attempted to derive the mean or SD based on available information (i.e., estimating the sample mean and SD from the sample size, median and/or interquartile range¹¹) or extract data from graphs using WebPlotDigitizer; ii) contacted the study authors by email to request additional data; or iii) excluded the study from the meta-analysis. Mean change from baseline and SD were retrieved for each group using the MA-cont:pre/post effect size interactive tool¹². The meta-analysis was conducted using R statistical software (version 4.2.0) with the metafor package,¹³ employing a random effects model. Results for the meta-analysis are displayed in forest plots. Two complementary methods were used to detect heterogeneity: visual inspection of the forest plot and numerical measures of heterogeneity (Cochran Q statistic and the inconsistency index (I²)).¹⁴ Heterogeneity was analysed with the I² statistic and interpreted as low <30%, 30%≤ moderate <50%, 50% ≤ substantial <75% or high ≥75%.¹⁵ The mean change between groups for each outcome measure was compared with the established minimal clinically important difference (MCID).

Risk of bias and quality of evidence assessment

Risk of bias was assessed using the Risk of Bias 2.0 tool.¹⁶ The Risk of Bias 2.0 tool assesses biases arising from the randomisation process (D1), deviations from the intended interventions (D2), missing outcome data (D3), measurement of the outcome (D4) and selection of the reported outcome (D5). Judgement can be ‘low’ or ‘high’ risk of bias or express ‘some concern’ and the overall risk of bias was reached using the signalling questions¹⁶. Risk of bias was assessed by one reviewer and checked by a second reviewer for accuracy. The Grading of Recommendations Assessment, Development, and Evaluation (GRADE)¹⁷ framework was used to assess the certainty of the evidence. Criteria for grading the level of certainty based on the GRADE guidelines are available in the supplementary material (Section A2). The GRADE assessment was performed separately by two independent reviewers, who then met to reach a consensus.

Results

Study characteristics

Eight studies^18–25 were included (Cohen’s Kappa = 0.61, substantial) and seven were considered for meta-analysis (Figure 1).

Figure 1.

Flow diagram of studies included on the effects of pulmonary rehabilitation on the functional status (capacity and performance) of adults with interstitial lung disease according to the preferred reporting items for systematic reviews and meta-analysis (PRISMA).

In total, 410 adults with ILD were included (mean age ranged from 59.1²⁵ to 71.0²² years; mean forced vital capacity from 52.4²⁵ to 76.0²⁴ percentage predicted; diffusing capacity for carbon monoxide from 36.6¹⁹ to 59.4²³ percentage predicted) (Table 1). Five studies^{18,19,21–23} included idiopathic pulmonary fibrosis (IPF) only, while three^20,24,25 involved mixed ILD populations. Studies were conducted in outpatient (n = 7)^18,20–25 or inpatient settings (n = 1)¹⁹. The duration of the PR programmes varied between 3¹⁹ and 26²⁰ weeks, with sessions lasting between 30 min²⁴ and 2 h.²² Inpatient PR sessions were conducted 5 to 6 times per week, while outpatient PR sessions were conducted 2 to 3 times per week. All studies included a combination of endurance and resistance training as well as education and/or psychosocial support. Educational sessions were conducted face-to-face; however, in seven studies, information was lacking regarding whether sessions were single or group-based, as well as details on delivery method, frequency, and/or duration of the educational component. Common topics of educational content were: nutrition,^{18–20,22,24} benefits of exercise and physical activity,^{18,19,21,22,24} anatomy and pathophysiology of chronic respiratory diseases,^18,22,24,25 dyspnoea and symptom management,^18,21,22,24 anxiety/depression/stress management,^18,20,22,24 and the role and correct use of medication/oxygen therapy.^18,22,24 All controls received usual care.

Table 1.

Overview of the characteristics and results of the included studies assessing the effects of pulmonary rehabilitation on the functional status of adults with interstitial lung disease (n = 8).

Study ID, Country	Setting, Length	Participants		Pulmonary rehabilitation		Results
Study ID, Country	Setting, Length	PR group	Control group	Exercise training	Education/psychosocial support	PR group	Control group
Dowman 2017, Australia²⁴	Outpatient, 8 weeks	N = 74 Age = 69.0±11.0 Sex (M/F) = 44/30 FVC (%pred) = 76.0±18.0 DLCO (%pred) = 50.0±16.0	N = 68 Age = 70.0±11.0 Sex (M/F) = 43/25 FVC (%pred) = 75.0±20.0 DLCO (%pred) = 48.0±14.0	Frequency: 2x/week Endurance training Modality: Cycling (cycle ergometer); walking (treadmill or ground-based) Intensity: Cycling - 70% WR (6MWT)/symptom-limited (mBorg 3-4); walking - 80% 6MWD/symptom-limited (mBorg 3-4) Progression: Walking - increased by 0.25 km/h (treadmill) or 62m (corridor walking) weekly for speeds <0.3 km/h, or by 0.5 km/h or 125m for speeds ≥3 km/h; upon reaching max speed, reduced by 0.2-0.4 km/h and added 1%–2% gradient; subsequent sessions increased gradient by 1%–2%; cycling - intensity was increased by 15% weekly Resistance training Modality: Free weights (dumbbells) Load: 10-12 repetition-max/symptom-limited (Borg 12-14) Progression: Lower limb resistance exercises were increased every 2 weeks by 1-3 kg/symptom-limited (Borg 12-14); Upper limb resistance exercises increased by 2-5 repetitions until a total of 20 repetitions/symptom-limited (Borg 12-14) Time: 30 min Volume: 60 min/week	Frequency: - Duration: - Type: - Topics: Understanding lung disease, respiratory function tests, medications, home oxygen therapy, self-management including exacerbations, managing breathlessness, energy conservation, exercise and physical activity, stress and anxiety, nutrition and healthy eating, sexuality and intimacy, community resources, swallowing, continence, and airway clearance	6MWT, m Pre: 477.0±107.0 Post:m 500.7±107.0	6MWT, m Pre: 441.0±142.0 Post: 439.0±142.0
Gaunaurd 2014, United States¹⁸	Outpatient, 12 weeks	N = 11 Age = 71.0±6.0 Sex (M/F) = NR FVC (%pred) = 60.0±11.0 DLCO (%pred) = 44.0±11.0	N = 10 Age = 66.0±7.0 Sex (M/F) = NR FVC (%pred) = 61.0±14.0 DLCO (%pred) = 43.0±11.0	Frequency: 2x/week Endurance training Modality: Walking (treadmill); cycling (recumbent cycling) Intensity: 70%–80% HR max Progression: - Resistance training Modality: Elastic resistance bands Load: Elastic resistance bands (yellow); 2 sets of 10 repetitions Progression: Sets and repetitions increased to 3 × 15, with higher resistance (green bands) Time: 75 min Volume: 150 min/week	Frequency: - Duration: - Type: - Topic(s): Medication, breathing exercises, exercise training, nutrition, pulmonary physiology, and psychological coping strategies	IPAQ, MET-min Pre: - Post: - MD: 51.4±57.7	IPAQ, MET-min Pre: - Post: - MD: 20.9±37.2
Jackson 2014, United States²²	Outpatient, 12 weeks	N = 11 Age = 71.0±6.0 Sex (M/F) = NR FVC (%pred) = 60.0±11.0 DLCO (%pred) = 44.0±11.0	N = 10 Age = 66.0±7.0 Sex (M/F) = NR FVC (%pred) = 61.0±14.0 DLCO (%pred) = 43.0±11.0	Frequency: 2x/week Endurance training Modality: Walking (treadmill); cycling (semi-recumbent cycling) Intensity: 60%–80% HR max Progression: - Resistance training Modality: Elastic resistance bands Load: Elastic resistance bands (yellow); 3 sets of 15 repetitions Progression: Increase in band resistance (yellow > red > green > blue) Time: 120 min Volume: 240 min/week	Frequency: Once every other week Duration: 15 min Type: Powerpoint presentations and handouts Topics: Medication use, breathing techniques, exercise strategies, proper nutrition, pulmonary physiology, and psychological coping mechanisms	6MWT, m Pre: 360.6±16.5 Post: 354.4±37.2	6MWT, m Pre: 339.4±34.4 Post: 324.1±35.5
Jarosch 2020, Germany¹⁹	Inpatient, 3 weeks	N = 34 Age = 68.0±9.0 Sex (M/F) = NR FVC (%pred) = 74.0±19.0 DLCO (%pred) = 44.1±15.4	N = 17Age = 65.0±10.0Sex (M/F) = NRFVC (%pred) = 72.0±20.0DLCO (%pred) = 36.6±18.8	Frequency: 5-6 days/week Endurance training Modality: Cycling (cycle ergometer), continuous or interval endurance training Intensity: 60%–100% WR max (CPET) – interval or continuous endurance training, if tolerated Progression: Symptom-limited (increase intensity/duration if mBorg <4) Resistance training Modality: - Load: 3 sets of 15-20 repetitions Progression: Symptom-limited (increase intensity/duration if mBorg <4) Time: - Volume: -	Frequency: - Duration: - Type: - Topics: Disease management, physical activity, nutritional counselling, and motivation	6MWT, m Pre: 415.0±101.0 Post: 469.0±139.5	6MWT, m Pre: 405.0±109.0 Post: 401.1±109.3
Nishiyama 2018, Japan²³	Outpatient, 10 weeks	N = 13 Age = 68.1±8.9 Sex (M/F) = 9/6 FVC (%pred) = 66.1±13.2 DLCO (%pred) = 59.4±16.7	N = 15 Age = 64.5±9.1 Sex (M/F) = 12/1 FVC (%pred) = 68.7±19.5 DLCO (%pred) = 48.6±16.7	Frequency: 2x/week Endurance training Modality: Cycling (cycle ergometer); walking (treadmill) Intensity: Cycling -80% WR max (CPET); walking -80% 6MWD Progression: - Resistance training Modality: Elastic resistance bands Load: - Progression: - Time: - Volume: -	Frequency: - Duration: - Type: - Topics: -	6MWT, m Pre: 385.0±116.0 Post: 427.0±84.0	6MWT, m Pre: 476.0±128.0 Post: 472.0±130.0
Perez-bogerd 2018, Belgium²⁰	Outpatient, 26 weeks	N = 30 Age = 64.0±13.0 Sex (M/F) = 22/8 FVC (%pred) = NR DLCO (%pred) = 41.0±13.0	N = 30 Age = 64.0±8.0 Sex (M/F) = 15/15 FVC (%pred) = NR DLCO (%pred) = 45.0±16.0	Frequency: 2-3x/week Endurance training Modality: Cycling (cycle ergometer); walking (treadmill); stair climbing Intensity: Cycling: 60% WR max (CPET); walking: 75% 6MWD; stair climbing: 2-min blocks (1-3 sets) Progression: Walking - 110% 6MWD; cycling - 85% WRmax; stair climbing - 3 sets of 2-min blocks(increments were symptom-limited until maximal targets reached) Resistance trainingModality: Machine weights Load: 70% 1RM; 3 sets of 8 repetitions Progression: - Time: 90 min Volume: 270 min/week	Frequency: - Duration: 30 min Type: - Topics: -	6MWT, m Pre: 462.0±57.0 Post: 504.0±57.0	6MWT, m Pre: 491.0±57.0 Post: 474.0±57.0
						Steps/day, steps Pre: 5671.0±2598.0 Post: 5721.0±2598.0	Steps/day, steps Pre: 7013.0±2598.0 Post: 6593.0±2598.0
						Time spent in moderate PA Pre: 36.0 [25.0; 52.0] Post: 37.0 [26.0; 54.0]	Time spent in moderate PA Pre: 54.0 [38.0; 78.0] Post: 44.0 [31-0; 64.0]
Vainshelboim 2014, Israel²¹	Outpatient, 12 weeks	N = 15 Age = 68.8±6.0 Sex (M/F) = 10/5 FVC (%pred) = 66.1±14.8 DLCO (%pred) = 48.6±17.2	N = 17 Age = 66.0±9.0 Sex (M/F) = 11/6 FVC (%pred) = 70.1±17.4 DLCO (%pred) = 53.2±12.2	Frequency: 2x/week Endurance training Modality: Cycling (cycle ergometer); walking (treadmill or ground-based) Intensity: Cycling - 50%–60% WR max (CPET)/symptom-limited (mBorg 3-5); walking - 70%–80% 6MWD/symptom-limited (mBorg 3-5) Progression: Cycling - 60%–70% WR max (CPET); walking - 80%–90% 6MWD Resistance training Modality: Free weights (dumbbells) Load: Symptom-limited (mBorg 3-5); 1 set of 12-15 repetitions Progression: Symptom-limited (mBorg 4-6); 2 sets of 10-12 repetitions Time: 60 min Volume: 120 min/week	Frequency: - Duration: - Type: - Topics: Managing symptoms and motivation to be physically active	6MWT, m Pre: 513.0±108.0 Post: 583.4±108.0	6MWT, m Pre: 471.0±108.0 Post: 460.40±108.0
Vainshelboim 2014, Israel²¹	Outpatient, 12 weeks					30-s STS, reps Pre: 11.5±2.8 Post: - MD: 3.7±2.6	30-s STS, reps Pre: 13.7±4.5 Post: - MD: −0.4±2.5
Ku 2017, India²⁵	Outpatient, 8 weeks	N = 20 Age = 59.1±10.4 Sex (M/F) = 8/12 FVC (%pred) = 52.4±13.7 DLCO (%pred) = NR	N = 20 Age = 62.1±14.5 Sex (M/F) = 8/12 FVC (%pred) = 58.6±13.7 DLCO (%pred) = NR	Frequency: 2x/week Endurance training Modality: Cycling (cycle ergometer); walking (not specified) Intensity: - Progression: - Resistance training Modality: Free weights Load: - Progression: - Time: 120 min Volume: 240 min/week	Frequency: - Duration: - Type: - Topics: Information about the disease, medications used in their treatment, how to change their behavior, and other non-drug treatments	6MWT, m Pre: 237.4±90.4 Post: 261.2±113.1	6MWT, m Pre: 208.0±93.7 Post: 211.4±108.8

Data are reported as mean ± SD and geometric mean [geometric 95% CI]. Legend: CPET, Cardiopulmonary exercise testing, DLCO, diffusing capacity of carbon monoxide, F, Female, FVC, Forced vital capacity, HR, Heart rate, IPF, idiopathic pulmonary fibrosis, IPAQ, International Physical Activity Questionnaire, MD, Difference in means, M, Male, mBorg, modified Borg scale, PA, Physical activity, PR, Pulmonary rehabilitation, WR, Work rate, 6MWT, Six-minute walk test, 1RM, One-repetition maximum, 30-s STS, 30-s sit-to-stand test.

Effects of pulmonary rehabilitation on functional status

Seven studies evaluated functional capacity using the 6-min walk test (6MWT) (n = 7)19–25 and one of these studies also used the 30-s sit-to-stand test (30-s STS) (n = 1).²¹ Two studies assessed functional performance, measuring time spent in moderate PA (n = 1),²⁰ and the number of steps per day with the SenseWear Armband (n = 1),²⁰ and energy expenditure in MET-min using the international physical activity questionnaire (IPAQ) (n = 1)¹⁸.

PR improved functional capacity measured with the 6MWT (MD 45.82 m, 95% CI [26.14; 65.50], I² = 71.54%, p < .001) (Figure 2) compared to usual care, which exceeded the MCID (29-34m).^26,27 The certainty level of evidence for the 6MWT was rated as low (Table 2). One study found a mean difference of 4.1 repetitions (PR: 3.7 ± 2.6 reps; control group: −0.4 ± 2.5 reps, p < .001) in the 30-s STS test²¹ in favour of the PR group, which is above the MCID of two repetitions.²⁸ There were no improvements in steps/day²⁰ or time spent in moderate PA²⁰ compared to the control group; self-reported PA¹⁸ (measured with the IPAQ) improved significantly after PR (PR: 51.4 ± 57.7 MET-min; control group: 20.9 ± 37.2 MET-min, p = .03). Risk of bias assessment for each outcome measure per study is in supplementary material (Section A3 – Table S1).

Figure 2.

Meta-analysis of the effects of pulmonary rehabilitation versus control group on the six-minute walk test in adults with interstitial lung disease (n = 7). The point estimate and the statistical size (proportional area of the square) are shown. Horizontal lines indicate 95% confidence intervals. The pooled mean difference was calculated using a random effects model.

Table 2.

Grading of recommendations, assessment, development and evaluations (GRADE) of the effects of pulmonary rehabilitation on the six-minute walk test in adults with interstitial lung disease (n = 7).

Number of studies	Study design	Risk of bias	Inconsistency	Indirectness	Imprecision	Other considerations	Absolut effect (95% CI)	Certainty
Seven	RCTs	No serious limitations	Substantial heterogeneity^a	Not detected	Inaccuracy detected^b	-	45.82 m, 95% CI [26.14; 65.50], I^b = 71.54%, p < .001	Low (⊕⊕⊕⊘)

Data are presented as mean difference [95% CI]. Legend: CG, Control group, EG, Experimental group, RCTs, Randomised controlled trials.

^aIf the p-value of the Cochran Q test is < 0.1, and the Higgins I2 statistic is ≥ 50%, statistically significant heterogeneity is assumed – Rate down one level.

^bFor continuous results, information is likely to be insufficient if the total number of participants is less than 400 – Rate down one level.

Discussion

This systematic review and meta-analysis demonstrated a positive effect of PR on functional capacity in people with ILD. Results for functional performance were inconclusive due to a lack of studies assessing this health domain.

The mean improvement in the 6MWT after PR was above the established MCID,²⁶ suggesting that PR leads to clinically meaningful changes in functional capacity in adults with ILD, consistent with findings from a recent Cochrane review.⁶ However, substantial heterogeneity was found, likely attributable to differences in the duration and frequency of sessions, exercise modality, and training intensities. Functional tests that reflect activities other than walking and are applicable in space-constrained settings were hardly ever considered. In fact, only one study used the 30-s STS, and it found significant improvements above the MCID²⁸ after PR. The inclusion of these tests is essential for a more comprehensive assessment of a person’s activity limitations.³

Functional performance was rarely assessed, and most measures were related to PA. The only reported improvement was in self-reported PA,¹⁸ but caution is warranted as this finding is based on just one study.²⁹ A large heterogeneity and limited number of studies hampered both the summary of results and our ability to draw further conclusions on functional performance. Future studies should focus on functional performance to provide information on the impact of PR on patients’ ability to participate in activities related to self-care, housekeeping, and social and recreational activities.

This systematic review exclusively includes studies adhering to the PR definition, requiring both exercise training and education.⁷ Often, the impact of education on functional status outcomes is underestimated, with primary focus placed on the exercise component. However, awareness of the disease and its impacts, improved medication use (including oxygen therapy), and symptom management (such as energy conservation and dyspnea relief), along with other topics covered during the educational component, are likely to influence and optimise patients’ functional capacity and performance. Education can empower patients to better adapt to their environment and manage their condition,³⁰ thereby promoting increased participation – effects that should not be dismissed. Nevertheless, there was often insufficient reporting on the education component, particularly concerning details such as session frequency and duration, delivery methods, session providers, and covered topics. These problems have also been observed in COPD studies and have been discussed elsewhere.³¹

The majority of included studies come from research on IPF, the most prevalent ILD.³² However, further research is needed for other ILDs, including exposure-related ILDs, connective tissue disease-associated ILDs, and fibrotic hypersensitivity pneumonitis. These conditions can lead to progressive pulmonary fibrosis,³³ negatively impacting functional status. We acknowledge that only peer-reviewed publications and randomised controlled trials were included. Additional interventional studies may exist in the unpublished grey literature, and good-quality non-randomised studies might have complemented the results obtained.

Conclusion

PR is effective at improving functional capacity and has been mostly measured with the 6MWT. Functional performance has been highly neglected in PR studies in adults with ILD, leading to limited and inconclusive findings. Future research should explore a wider range of activities and assess the effectiveness of PR in improving patients' daily lives, focusing on what they actually do rather than just what they are capable of doing.

Supplemental Material

Supplemental Material - Functional status following pulmonary rehabilitation in people with interstitial lung disease: A systematic review and meta-analysis

Supplemental Material for Functional status following pulmonary rehabilitation in people with interstitial lung disease: A systematic review and meta-analysis by Guilherme Rodrigues, Rute Santos, Rita Pinto, Ana Oliveira, and Alda Marques in Chronic Respiratory Disease

Footnotes

Declaration of conflicting interests

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The author(s) received no financial support for the research, authorship, and/or publication of this article.

ORCID iDs

Alda Marques

Guilherme Rodrigues

Supplemental Material

Supplemental material for this article is available online.

References

Tremblay Labrecque

P-F

Dion

Saey

. Functional clinical impairments and frailty in interstitial lung disease patients. ERJ Open Research 2022; 8: 00144–02022. DOI: 10.1183/23120541.00144-2022.

Aronson

Danoff

Russell

, et al. Patient-centered outcomes research in interstitial lung disease: an official American thoracic society research statement. Am J Respir Crit Care Med 2021; 204: e3–e23. DOI: 10.1164/rccm.202105-1193ST.

Bui

Nyberg

Maltais

, et al. Functional tests in chronic obstructive pulmonary disease, Part 1: clinical Relevance and links to the international classification of functioning, disability, and health. Ann Am Thorac Soc 2017; 14: 778–784. DOI: 10.1513/AnnalsATS.201609-733AS.

Lareau

Blackstock

. Functional status measures for the COPD patient: a practical categorization. Chron Respir Dis 2019; 16: 1479973118816464. DOI: 10.1177/1479973118816464.

Iwakura

Kawagoshi

Tamaki

, et al. Physical activity measurements in individuals with interstitial lung disease: a systematic review and meta-analysis. Eur Respir Rev 2023; 32: 20230712. DOI: 10.1183/16000617.0165-2022.

Dowman

Hill

May

, et al. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database Syst Rev 2021; 2: Cd006322. DOI: 10.1002/14651858.CD006322.pub4.

Spruit

Singh

Garvey

, et al. An official American Thoracic Society/European Respiratory Society statement: key concepts and advances in pulmonary rehabilitation. Am J Respir Crit Care Med 2013; 188: e13–e64. DOI: 10.1164/rccm.201309-1634ST.

Alsina-Restoy

Torres-Castro

Caballería

, et al. Pulmonary rehabilitation in sarcoidosis: a systematic review and meta-analysis. Respir Med 2023; 219: 107432. DOI: 10.1016/j.rmed.2023.107432.

Page

McKenzie

Bossuyt

, et al. The PRISMA 2020 statement: an updated guideline for reporting systematic reviews. Bmj 2021; 372: n71. DOI: 10.1136/bmj.n71.

10.

Landis

Koch

. The measurement of observer agreement for categorical data. Biometrics 1977; 33: 159–174.

11.

Wan

Wang

Liu

, et al. Estimating the sample mean and standard deviation from the sample size, median, range and/or interquartile range. BMC Med Res Methodol 2014; 14: 135. DOI: 10.1186/1471-2288-14-135.

12.

Papadimitropoulou

Riley

Dekkers

, et al. MA-cont:pre/post effect size: an interactive tool for the meta-analysis of continuous outcomes using R Shiny. Res Synth Methods 2022; 13: 649–660. DOI: 10.1002/jrsm.1592.

13.

Viechtbauer

. Conducting meta-analyses in R with the metafor package. J Stat Software 2010; 36: 1–48. DOI: 10.18637/jss.v036.i03.

14.

Cordero

Dans

. Key concepts in clinical epidemiology: detecting and dealing with heterogeneity in meta-analyses. J Clin Epidemiol 2021; 130: 149–151. DOI: 10.1016/j.jclinepi.2020.09.045.

15.

Deeks

Higgins

Altman

, et al. Analysing data and undertaking meta-analyses. Cochrane Handbook for Systematic Reviews of Interventions. 2019; 241–284.

16.

Sterne

JAC

Savović

Page

, et al. RoB 2: a revised tool for assessing risk of bias in randomised trials. Bmj 2019; 366: 1–8. DOI: 10.1136/bmj.l4898.

17.

Guyatt

Oxman

Schünemann

, et al. GRADE guidelines: a new series of articles in the Journal of Clinical Epidemiology. J Clin Epidemiol 2011; 64: 380–382. DOI: 10.1016/j.jclinepi.2010.09.011.

18.

Gaunaurd

Gómez-Marín

Ramos

, et al. Physical activity and quality of life improvements of patients with idiopathic pulmonary fibrosis completing a pulmonary rehabilitation program. Respir Care 2014; 59: 1872–1879. DOI: 10.4187/respcare.03180.

19.

Jarosch

Schneeberger

Gloeckl

, et al. Short-Term effects of comprehensive pulmonary rehabilitation and its maintenance in patients with idiopathic pulmonary fibrosis: a randomized controlled trial. J Clin Med 2020; 9: 1567. DOI: 10.3390/jcm9051567.

20.

Perez-Bogerd

Wuyts

Barbier

, et al. Short and long-term effects of pulmonary rehabilitation in interstitial lung diseases: a randomised controlled trial. Respir Res 2018; 19: 182. DOI: 10.1186/s12931-018-0884-y.

21.

Vainshelboim

Oliveira

Yehoshua

, et al. Exercise training-based pulmonary rehabilitation program is clinically beneficial for idiopathic pulmonary fibrosis. Respiration 2014; 88: 378–388. DOI: 10.1159/000367899.

22.

Jackson

Gómez-Marín

Ramos

, et al. Exercise limitation in IPF patients: a randomized trial of pulmonary rehabilitation. Lung 2014; 192: 367–376. DOI: 10.1007/s00408-014-9566-9.

23.

Nishiyama

Kondoh

Kimura

, et al. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology 2008; 13: 394–399. DOI: 10.1111/j.1440-1843.2007.01205.x.

24.

Dowman

McDonald

Hill

, et al. The evidence of benefits of exercise training in interstitial lung disease: a randomised controlled trial. Thorax 2017; 72: 610–619. DOI: 10.1136/thoraxjnl-2016-208638.

25.

Vivek

Janmeja

Aggarwal

, et al. Pulmonary rehabilitation in patients with interstitial lung diseases in an outpatient setting: a randomised controlled trial. Indian J Chest Dis Allied Sci 2017; 59: 75–80.

26.

Holland

Hill

Conron

, et al. Small changes in six-minute walk distance are important in diffuse parenchymal lung disease. Respir Med 2009; 103: 1430–1435. DOI: 10.1016/j.rmed.2009.04.024.

27.

Holland

Nici

. The return of the minimum clinically important difference for 6-minute-walk distance in chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2013; 187: 335–336. DOI: 10.1164/rccm.201212-2191ED.

28.

Zanini

Crisafulli

D'Andria

, et al. Minimum clinically important difference in 30-s sit-to-stand test after pulmonary rehabilitation in subjects with COPD. Respir Care 2019; 64: 1261–1269. DOI: 10.4187/respcare.06694.

29.

Lee

Macfarlane

Lam

, et al. Validity of the international physical activity questionnaire short form (IPAQ-SF): a systematic review. Int J Behav Nutr Phys Act 2011; 8: 115. DOI: 10.1186/1479-5868-8-115.

30.

Lee

JYT

Tikellis

Dowman

, et al. Self-management interventions for people with pulmonary fibrosis: a scoping review. Eur Respir Rev 2023; 32: 230092. DOI: 10.1183/16000617.0092-2023.

31.

Roberts

Kidd

Kirkwood

, et al.

How is the education component of pulmonary rehabilitation delivered in practice--Is it patient-centred?

Clin Respir J 2021; 15: 835–842. DOI: 10.1111/crj.13371.

32.

Kaul

Cottin

Collard

, et al. Variability in global prevalence of interstitial lung disease. Front Med 2021; 8: 751181. DOI: 10.3389/fmed.2021.751181.

33.

Rajan

Cottin

Dhar

, et al. Progressive pulmonary fibrosis: an expert group consensus statement. Eur Respir J 2023; 61: 2103187. DOI: 10.1183/13993003.03187-2021.

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