Our community is important to us. We acknowledge the value of the opinions of people affected by the disease, their families and carers, and all healthcare professionals involved in the care of progressive pulmonary fibrosis (PPF). For this reason, we partnered with the James Lind Alliance and the charity Action For Pulmonary Fibrosis to identify the area on which the research should focus, according to stakeholders' preferences.

We set up a Priority Setting Partnership (PSP). The PSP took nearly two years to complete and involved people with lived experience of pulmonary fibrosis and health care professionals throughout the process.

With the first survey, we asked stakeholders to share their uncertainties about diagnosis, treatment, and management of progressive pulmonary fibrosis research. Over 600 people responded with more than 2500 single questions and statements! Responses were coded to identify recurrent themes, from which summary research questions were generated. We then verified that those themes had not already been thoroughly investigated. After evidence review, 44 questions were evaluated as not-answered and included in the second survey. Approximately 800 people completed the survey and selected their favourites. Finally, the 15 highly top-rated questions were ranked during a two-day workshop to select the top ten.

We highlighted three main interest areas:

  • We should aim for a quicker, more accurate diagnosis, with improved services in primary care
  • We should research new treatments and assess the efficacy of drugs and non-drug interventions to improve our patients' quality and length of life.
  • Symptoms management. Cough, phlegm and breathlessness impact the quality of life deeply.

Deciding upon the final top ten research priorities was challenging, and questions not included in the top ten remain very important to be answered. We will release the complete list soon. Further information can be found at (APF website and JLA website)

The Top Ten Progressive Pulmonary Research Priorities:

  1. How can the diagnosis of PPF (Progressive Pulmonary Fibrosis) be improved in terms of accuracy and the time taken (screening programme, early signs and symptoms that could be detected in primary care, blood markers, imaging, biopsy, artificial intelligence, etc.)?
  2. Can new treatments other than pirfenidone and nintedanib slow, halt or reverse the progression of PPF?
  3. What can be done to improve the speed and accuracy of PPF diagnosis in primary care (e.g. training, integration of case-based studies in GP training, awareness campaigns)?
  4. What is the best time for drug and non-drug interventions (pulmonary rehab, oxygen therapy, psychological support) to start to preserve quality and length of life for patients with PPF?
  5. What are the best ways (drug, non-drug and aids) to treat cough in PPF?
  6. Would early treatment delay progression, lung function decline, and improve survival in PPF?
  7. Which therapies will improve survival in PPF?
  8. What treatments (drug, non-drug and aids) can reduce breathlessness and phlegm production in PPF?
  9. To what extent do different interventions (pulmonary rehab, oxygen therapy, psychological support) impact length of life in patients with PPF?
  10. Can new treatments for PPF be developed with reduced side effects? Does how the drug is delivered (e.g. oral, nebulised, through a vein) affect potential side effects of the drug in PPF?