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  • Cystic Fibrosis

Key Highlights From ECFS 2023: JLA Top 10 Research Priorities

Historically, Cystic Fibrosis has been dominated by repeated cycles of infection and inflammation leading to irreparable structural lung disease.1

During the last 2 years, 90% of people with CF in the UK have become eligible for treatment with CFTR modulators.2 Patients taking these medicines have reported clinical benefits such as improved lung function and a reduction in exacerbations.3,4

However, modulators are unlikely to repair the already structurally damaged lung, and their effects on the lung microbiome and their potential for reducing inflammation is not yet fully understood.5,6

In 2022 the James Lind Alliance (JLA) Priority Setting Partnership (PSP) in Cystic Fibrosis published its refreshed list of research priorities.7

Priority areas identified in the refresh exercise included managing an aging CF population; managing the effects of CFTR modulators on systems outside the lung; long term safety of CF treatments including CFTR modulators: diagnosing infection in the absence of sputum as well as what the options are for patients unable to take modulators or for whom there are currently no novel therapies available.7

There are still many unknowns in this fast-changing CF landscape for both clinicians and patients, as such the need for research remains a high priority in this field.

In this presentation, Dr Freddy Frost summarise some of the data from ECFS 2023 aligned with the JLA Top 10 research priorities for CF.


1. Elborn, J Stuart. Cystic fibrosis. The Lancet 2016; 388(10059): 2519–2531.

2. CF trust registry report 2021. Available at: https://www.cysticfibrosis.org.uk/sites/default/files/2022-10/CFT_2021-Annual-Data-Report-WEB.pdf

3. Middleton PG et al. N Engl J Med 2019; 381(19):1809–19.

4. Heijerman HGM et al. The Lancet 2019; 394(10212):1940–8.

5. Keone et al. Int. J. Mol. Sci. 2020, 21, 6379.

6. Harvey et al. Int. J. Mol. Sci. 2022, 23, 3513.

7. James Lind Alliance CF priorities. Available at: https://www.jla.nihr.ac.uk/priority-setting-partnerships/cystic-fibrosis-refresh/

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  • Dr Freddy Frost

    NIHR Academic Clinical Lecturer

    Read biography