Cystic fibrosis liver disease progression in the era of elexacaftor–tezacaftor–ivacaftor
In this nationwide French cohort study of over 10,000 people with cystic fibrosis, we observed a significant decline in the incidence of cystic fibrosis liver disease outcomes following the rollout of elexacaftor-tezacaftor-ivacaftor (ETI) in December 2019. The rate dropped from 25.4 to 1.2 per 1,000 person-years post-ETI. Matched analyses confirmed a reduced risk of liver disease progression, with an adjusted incidence rate ratio of 0.28. Although the findings suggest ETI may improve liver outcomes in people with cystic fibrosis, potential confounding factors necessitate further research to establish causality and understand the underlying biological mechanisms.
Authors
- Philippe Sogni (ORCID: https://orcid.org/0000-0003-3316-8785)
- Vincent Olivier Mallet (ORCID: https://orcid.org/0000-0003-2219-9201)
- Valérie Dhalluin-Venier
- Stanislas Pol
- Anaïs Vallet‐Pichard (ORCID: https://orcid.org/0000-0001-9728-3913)
- Charlotte Mouliade (ORCID: https://orcid.org/0000-0001-5698-9936)
- Samir Bouam (ORCID: https://orcid.org/0000-0002-2406-2391)
- Lucia Parlati (ORCID: https://orcid.org/0000-0003-1939-5607)
- Stylianos Tzedakis (ORCID: https://orcid.org/0000-0002-5934-0320)
- Pierre‐Régis Burgel (ORCID: https://orcid.org/0000-0003-0903-9828)
- Mathis Collier (ORCID: https://orcid.org/0009-0000-2398-0396)
- Reem Kanaan
Institutions
- Université Paris Cité (FR)
- Assistance Publique – Hôpitaux de Paris (FR)
- Maternité Port Royal (FR)
- Groupe Hospitalier Cochin - Port-Royal, Hôtel-Dieu, Broca - La Collégiale (FR)
Publication Details
- Journal
- JHEP Reports
- Published
- 2025-07-05
- DOI
- https://doi.org/10.1016/j.jhepr.2025.101512
- Citations
- 1
- Primary Topic
- Cystic Fibrosis Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 1.52