The Top Ten Priorities for PPF Research are published!!

Earlier in the year, we asked for your help to generate a list of the top ten priorities for PPF (progressive pulmonary fibrosis) research. This list is way of communicating to the research world what you as the people affected by pulmonary fibrosis would like us to focus on.

Through the partnership of the James Lind Alliance, Imperial College London and a Steering Committee including patients, carers, family members and clinicians, a list of 1,600 questions relating to progressive pulmonary fibrosis research was generated. The questions were narrowed down to top ten priorities which will guide years of research to come. These were recently published by the Action for Pulmonary Fibrosis charity and are outlined below.

We are excited to share that the Top Ten Progressive Pulmonary Research Priorities are:

  1. Improved diagnosis: 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. Novel treatments: Can new treatments other than Pirfenidone and Nintedanib slow, halt or reverse the progression of PPF?
  3. Improved awareness: 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. Timing of treatments: 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. Cough: What are the best ways (drug, non-drug and aids) to treat cough in PPF?
  6. Benefits of early treatment: Would early treatment delay progression, lung function decline, and improve survival in PPF?
  7. Improving survival – medications: Which therapies will improve survival in PPF?
  8. Breathlessness and phlegm: What treatments (drug, non-drug and aids) can reduce breathlessness and phlegm production in PPF?
  9. Improving survival – non pharmacological treatments: To what extent do different interventions (pulmonary rehab, oxygen therapy, psychological support) impact length of life in patients with PPF?
  10. Drug delivery for reduced side-effects: 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?

Firstly, we would like to acknowledge the incredible work of the Steering Committee, Action for Pulmonary Fibrosis team, the James Lind Alliance and Imperial College for their work generating this list. It was a huge feat of logistics and hard work to create a focused list in a complex medical field with many unanswered questions.

Secondly, but no less importantly, we would like to thank YOU. Thank you so much to all of you who participated in the survey. Your contributions will shape the future landscape of progressive pulmonary fibrosis research and will impact the lives of people affected by this condition for years to come.

What are we doing here at Breathing Matters…

The priorities address the need for

  1. Treatments (pharmacological and non-pharmacological): These need to be assessed for their ability to
    • Target FIBROSIS or the main symptoms of COUGH, MUCUS production, and SHORTNESS of breath.

and ultimately whether they

  • Improve both QUALITY and QUANTITY of life in patients with pulmonary fibrosis.
  1. Personalised Medicine: the RIGHT treatment, at the RIGHT time for an individual patient.


Our team of researchers are working on the priorities outlined above. Our projects include:

  • Novel Biomarkers – either from a simple blood test or a special imaging technique to:
    • Pick up early PF.
    • Understand how an individual patient is responding to treatment.
    • Identify patients that may respond to specific treatments but not others.
    • We have some fascinating data looking at mucin MUC1 that is abnormally presented in the lung in PPF and can be found in the blood of patients with PPF. We are developing a robust blood test to measure the levels of this mucin.
  • Better treatments: One of the most challenging aspects of managing patients with PPF are our limited treatment options. Whilst the anti-fibrotic medications Nintedanib and Pirfenidone are shown to slow the decline of lung function in PPF, they do not cure, nor even reverse the progression of PPF and patients may develop limiting gastrointestinal side effects from repeated oral medication:
    • Inhaled therapies: these have the potential to reach the fibrotic lung with fewer side effects and we have the first data on this approach in PF. 
    • New treatments with fewer/no side-effects: Our novel compound C21 is showing promising results in interim testing. Read more here. 
    • Our award winning work on neutrophil extracellular traps in PF and COVID-19 has potentially identified a novel drug target
    • Treating cough and mucin production: we are assessing the ability of inhaled therapies to reduce mucus in PF and assess the impact on cough


If you would like to know more about how the top 10 priority list was generated and how it might impact you, read on…

How the list was generated – Firstly, a steering committee was established including patients, carers and clinicians to help guide the project. An initial survey was made asking people affected by PPF and healthcare workers what the main unanswered questions in PPF were. Over 600 people responded with over 2000 questions and statements!  A second survey then helped to shortlist the questions into order of importance. Lastly, a two day workshop consisting of a team of people affected by PPF and healthcare workers finalized the top ten priorities in PPF research.

What happens next? – These priorities will be shared to the research world through social media, publication in journal articles, presentations at conferences. These priorities will not only help to allocate funding, but will also direct campaigning and awareness raising. It will identify gaps for future research and facilitate better communication between those affected by PPF and the scientific world.

For full article link see here.


Here at Breathing Matters, we are confident that with your support we can make substantial progress on achieving these aims in areas that are of importance to patients, their relatives, and their clinical teams.

Thank you all again for your important contributions.  If you would like to donate towards our work outlined above, you can do so here

Together we are stronger because …  Breathing Matters.



[Posted 11.7.22]

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