Progressive Pulmonary Fibrosis
About this PSP
The notion of the Progressive Pulmonary Fibrosis JLA PSP was originally developed by Professor Gisli Jenkins and supported by leading national charity Action for Pulmonary Fibrosis. Progressive pulmonary fibrosis represents the chronic evolution of a series of rare pulmonary conditions, commonly known as interstitial lung diseases (ILDs). They include idiopathic pulmonary fibrosis, non-specific interstitial pneumonia, chronic hypersensitivity pneumonitis, unclassifiable ILD, connective tissue disease-associated ILD, sarcoidosis, and occupational disease related ILD. These diseases are often underdiagnosed, and their exact prevalence is not known. Idiopathic pulmonary fibrosis, the best known among the ILDs, is estimated to affect around 32,500 people in the UK, while sarcoidosis around 6,600.
The aim of the progressive pulmonary fibrosis PSP was to identify the unanswered questions about progressive pulmonary fibrosis, from patient, carer and clinical perspectives, and then prioritise those that patients, carers and clinicians agree are the most important for research to address. The scope included questions about diagnosis, and also treatment and management of the disease (daily living at every stage) before and after the diagnosis.
The Progressive Pulmonary Fibrosis PSP Top 10 was published in June 2022.
The image below shows the PSP Steering Group at their first meeting on 22 October 2020.
Top 10 priorities
- 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.)?
- Can new treatments other than pirfenidone and nintedanib slow, halt or reverse the progression of PPF?
- 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)?
- 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?
- What are the best ways (drug, non-drug and aids) to treat cough in PPF?
- Would early treatment delay progression, lung function decline, and improve survival in PPF?
- Which therapies will improve survival in PPF?
- What treatments (drug, non-drug and aids) can reduce breathlessness and phlegm production in PPF?
- To what extent do different interventions (pulmonary rehab, oxygen therapy, psychological support) impact length of life in patients with PPF?
- 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?