2 Research recommendations

The Guideline Development Group has made the following recommendations for research, based on its review of evidence, to improve NICE guidance and patient care in the future. The Guideline Development Group's full set of research recommendations is detailed in the full guideline.

2.1 Pulmonary rehabilitation to improve outcomes in people with idiopathic pulmonary fibrosis

Does pulmonary rehabilitation improve outcomes for people with idiopathic pulmonary fibrosis?

Why this is important

There is evidence that people with idiopathic pulmonary fibrosis may benefit from pulmonary rehabilitation. However this evidence is mostly derived from programmes designed principally for people with chronic obstructive pulmonary disease. It is likely that the needs of people with idiopathic pulmonary fibrosis and chronic obstructive pulmonary disease differ. Randomised controlled trials should be carried out to determine the effects of pulmonary rehabilitation programmes tailored to idiopathic pulmonary fibrosis, compared with currently offered pulmonary rehabilitation programmes, on quality of life, walking distance and lung function with analysis adjusting for confounding factors appropriately. Trials should analyse benefits of the different aspects of pulmonary rehabilitation including the components, setting and location of the programme, and healthcare resources involved. End points may include: 6‑minute walk distance; breathlessness score; a measure of health-related quality of life (ideally employing a tool validated in people with idiopathic pulmonary fibrosis); mortality (all-cause and idiopathic pulmonary fibrosis-related); hospitalisation (all-cause, non-elective and idiopathic pulmonary fibrosis -related); lung function (vital capacity and diffusion capacity for carbon monoxide). Studies should be of sufficient power and duration and include a health economic evaluation.

2.2 Ambulatory oxygen to improve outcomes in idiopathic pulmonary fibrosis

Does ambulatory oxygen improve outcomes in idiopathic pulmonary fibrosis?

Why this is important

People with idiopathic pulmonary fibrosis frequently demonstrate a fall in oxygen saturation during exercise even though they are not hypoxic at rest. In such people, ambulatory oxygen is often provided to improve exercise capacity, enhance mobility and enable activities of daily living in order to improve quality of life. However, there are no randomised controlled trials to demonstrate that ambulatory oxygen therapy is effective in achieving these aims in patients with idiopathic pulmonary fibrosis. A randomised controlled trial should be conducted to determine the effects of ambulatory oxygen on quality of life in people with idiopathic pulmonary fibrosis and consideration given to the use of a placebo arm. This should include a standardised protocol for assessing exercise such as the 6-minute walk test. The end points may include 6‑minute walk distance; breathlessness score; a measure of health-related quality of life (ideally employing a tool validated in idiopathic pulmonary fibrosis patients). Phase III trials should have a duration of greater than 12 months and include a health economic evaluation.

2.3 Anti-reflux therapy as a treatment for idiopathic pulmonary fibrosis

Is anti-reflux therapy an effective treatment for idiopathic pulmonary fibrosis?

Why this is important

There is evidence from observational studies, and uncontrolled interventional trials, that microaspiration of gastric/oesophageal contents contribute to disease progression, and perhaps even cause idiopathic pulmonary fibrosis. There have been no randomised controlled trials of anti-reflux therapy in idiopathic pulmonary fibrosis but proton-pump inhibitors are often prescribed for symptoms of acid-reflux. A randomised, placebo-controlled trial of adequate power and duration of greater than 12 months should be undertaken to determine the benefits and side effects of anti-reflux therapy, including proton pump inhibition in people with a confirmed diagnosis of idiopathic pulmonary fibrosis. Appropriate end points may include mortality (all-cause and idiopathic pulmonary fibrosis-related); hospitalisation (all-cause, non-elective and idiopathic pulmonary fibrosis-related); lung function (vital capacity and diffusion capacity for carbon monoxide); 6‑minute walk distance; breathlessness score; a measure of health-related quality of life (ideally employing a tool validated in idiopathic pulmonary fibrosis patients). Phase III trials should include a health economic evaluation.

  • National Institute for Health and Care Excellence (NICE)