Extract
Elexacaftor-tezacaftor-ivacaftor (ETI) is a combination of small molecules that partially restore cystic fibrosis transmembrane conductance (CFTR) function, improving chloride and bicarbonate transport in epithelia in selected people with cystic fibrosis (pwCF). In Europe, pwCF carrying at least one F508del CFTR variant and aged ≥6 years are eligible to ETI, corresponding to approximately 85% of pwCF. Clinical benefits associated with ETI treatment include improvement in respiratory symptoms, increase in lung function and weight and decreased rates of pulmonary exacerbations [1, 2]. Importantly these effects are also observed in patients with advanced lung disease [3]. However, ETI may not result in improvement in all aspects of CF; for example, previous studies have shown that the highly effective CFTR modulator ivacaftor is unable to fully eradicate chronic airway bacterial infection [4]. Further, little data is currently available on the impact of CFTR modulators, including ETI, on inflammatory markers and even less data is available in the subset of patients with advanced lung disease, who are usually excluded from clinical trials.
Footnotes
This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.
Conflict of Interest: Clémence Martin reports lecture honoraria from Astra Zeneca, Chiesi, Zambon; travel support from Zambon, Sanofi, advisory board participation with Vertex, Zambon, GSK; outside the submitted work.
Conflict of Interest: Pierre-Régis Burgel reports grants from Boehringer Ingelheim, GSK, Vertex; consulting fees and lecture honoraria from Boehringer Ingelheim, GSK, AstraZeneca, Vertex, Chiesi, Pfizer, Novartis, Zambon, Insmed; outside the submitted work.
Conflict of Interest: All other authors have nothing to disclose.
- Received August 30, 2022.
- Accepted November 19, 2022.
- Copyright ©The authors 2022. For reproduction rights and permissions contact permissions{at}ersnet.org
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