People with idiopathic pulmonary fibrosis who received pulmonary rehabilitation could walk 44 metres further in six minutes than those who did no exercise. Quality of life also improved.
Pulmonary fibrosis is a rare condition where scar tissue builds up in the lungs making them stiff and causing breathing difficulty. The term idiopathic means there is no known cause. It tends to get worse over time, reducing a person’s activity levels.
Pulmonary rehabilitation is a core part of care, but most programmes were based on evidence in people with chronic obstructive pulmonary disease which is the commonest lung condition. This review looked at whether it improved outcomes for pulmonary fibrosis.
Only five small trials were available, and there are some concerns about the quality of evidence. However, this may be the best evidence attainable. Programme content varied making it difficult to know which exercises help most and it’s unknown whether it affects prognosis.
The review supports the use of pulmonary rehabilitation as currently recommended.
Why was this study needed?
Data from 2012 indicated that 32,500 people in the UK had idiopathic pulmonary fibrosis. This is an increase on previous estimates, which could be due to improved recording of the condition. It mostly affects men over 40 and is more common in North-West England, Scotland, Wales and Northern Ireland. Average survival following diagnosis is only three years.
There are two drugs approved to treat idiopathic pulmonary fibrosis, pirfenidone and nintedanib. They can slow progression but do not reverse it. Rarely people will be suitable for lung transplant. Otherwise care is supportive, centring upon oxygen therapy and pulmonary rehabilitation. But to date, there has been little clear evidence that pulmonary rehabilitation improves outcomes.
This systematic review aimed to pool results from all available trials assessing whether pulmonary rehabilitation improves exercise tolerance and quality of life in people with idiopathic pulmonary fibrosis.
What did this study do?
The review and meta-analysis identified five randomised controlled trials comparing pulmonary rehabilitation with usual care in 130 people with idiopathic pulmonary fibrosis. Trials were small with sample sizes ranging from 21 to 32. Average participant age was 67 years, and most participants were male.
Pulmonary rehabilitation differed across trials, variably including treadmill training, cycling, flexibility and strength training, often performed at around 50-80% maximum heart rate. Programmes typically involved 60-90 minute sessions given twice weekly for between 10 and 12 weeks.
Trials were published from 2008 to 2015 and were of moderate quality. None were from the UK. In all studies, participants and assessors were aware of treatment given. Only a single trial analysed all participants in their assigned group regardless of whether they’d completed treatment.
What did it find?
- People who received pulmonary rehabilitation were able to walk 44 metres further on the six-minute walk test than those having usual care (95% confidence interval [CI] +5.3 to +82.8 metres; four trials, 113 people).
- Pulmonary rehabilitation improved quality of life on the 50-item St George’s Respiratory Questionnaire, where a score change of four or more is considered clinically meaningful (mean difference [MD] -7.4, 95% CI -10.7 to -4.1; four trials, 113 people). By specific questionnaire section, rehabilitation improved symptoms (MD -18.5, 95% CI -26.9 to -10.1) and psychosocial impact (MD -9.0, 95% CI -11.6 to -6.4). There was no meaningful effect on activity levels (MD -1.25, 95% CI -1.25 to +0.11).
- No studies reported any side effects of pulmonary rehabilitation.
What does current guidance say on this issue?
The NICE guideline on idiopathic pulmonary fibrosis recommends that need for pulmonary rehabilitation is assessed at the time of diagnosis, usually by the six-minute walk test and quality of life assessment. Assessments should be repeated at six- to 12-monthly intervals. There is a specific recommendation for rehabilitation if the person is breathless upon exertion, but not if at rest.
If indicated, pulmonary rehabilitation is advised to include exercise and educational components, and be tailored to the needs of the individual.
Separate NICE technical guidelines outline the specific patient eligibility criteria for the use of pirfenidone and nintedanib.
What are the implications?
At the time of their last guideline, NICE recommended a need for research into whether pulmonary rehabilitation improves outcomes in idiopathic pulmonary fibrosis.
The findings of this review are positive but show that the evidence remains quite limited. To provide effective and individually-tailored programmes for people with idiopathic pulmonary fibrosis, there is still need for continued research and follow-up.
There is need to understand better the optimal content, intensity and duration of rehabilitation programmes. There is also need to look to the longer term and see whether it improves lung function and survival for this condition with known poor prognosis.
Citation and Funding
Gomes-Neto M, Silva CM, Ezequiel D, et al. Impact of Pulmonary Rehabilitation on Exercise Tolerance and Quality of Life in Patients With Idiopathic Pulmonary Fibrosis: A SYSTEMATIC REVIEW AND META-ANALYSIS. J Cardiopulm Rehabil Prev. 2018. [Epub ahead of print].
No funding was involved in the development of this systematic review.
British Lung Foundation. Idiopathic pulmonary fibrosis statistics. London: British Lung Foundation.
NICE. Idiopathic pulmonary fibrosis in adults: diagnosis and management. CG163. London: National Institute for Health and Care Excellence; 2013, updated 2017.
NICE. Pirfenidone for treating idiopathic pulmonary fibrosis. TA504. London: National Institute for Health and Care Excellence; 2018.
NICE. Nintedanib for treating idiopathic pulmonary fibrosis. TA379. London: National Institute for Health and Care Excellence; 2016.
The Pulmonary Fibrosis Trust. About pulmonary fibrosis. Staffordshire: The Pulmonary Fibrosis Trust.
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