Research priorities for pulmonary fibrosis should focus on developing medications to reverse scarring in the lungs and to improve lung function, according to an Australian survey.

Results from a poll of almost 200 people with pulmonary fibrosis, caregivers and healthcare professionals as well as researchers involved with pulmonary fibrosis have identified 10 top priorities for future research.

The responses elicited by Monash University researchers, showed that a high priority was also placed on research into ways of preventing pulmonary fibrosis and interventions aimed at alleviating symptoms.

There was good consensus among all respondents on the top 10 priorities, although ,healthcare professional and researchers tended to prioritise research into causes of acute exacerbations and early diagnosis for improving survival.

Published in Thorax, the top 10 research priorities were:

1. What medications can reverse scarring in the lungs of people with pulmonary fibrosis?
2. What medications can improve lung function in people with pulmonary fibrosis?
3. What are the causes of pulmonary fibrosis?
4. How can we prevent pulmonary fibrosis?
5. What are the most effective interventions to reduce shortness of breath in people with pulmonary fibrosis?
6. What medications can reduce symptoms and improve well-being in pulmonary fibrosis?
7. What are the most effective interventions to reduce cough in people with pulmonary fibrosis?
8. What medications can stop pulmonary fibrosis getting worse over time?
9. Can we develop anti-fibrotic drugs for pulmonary fibrosis that are equally effective as current options but with fewer side effects?
10. What is the best exercise programme for people with pulmonary fibrosis?

The authors of the paper said there was a need to prioritise research into pulmonary fibrosis because although antifibrotic therapies slow down the decline in lung function, they have no beneficial effect on the debilitating symptoms and impaired quality of life.

“Therefore, most people with pulmonary fibrosis continue to experience a high symptom burden, a progressive decline in lung function and a reduced physical capacity that negatively impacts their physical, psychological and social well-being,” they wrote.

They noted that several themes proposed for pulmonary fibrosis research, such as focusing on rapid and accurate diagnosis, improving education/awareness of the disease, better psychosocial support and self-management, were not ranked in the final top 10.

“This is the first research setting exercise for pulmonary fibrosis. The results will allow researchers, funders and policymakers to ensure that future research efforts are well aligned with stakeholder priorities,” they concluded.