Long-term impact of elexacaftor/tezacaftor/ivacaftor on small and large airways in people with cystic fibrosis aged ≥6 years: 24-month real-world evidence from the German Cystic Fibrosis Registry
Our real-world data showed significant improvements in lung function, including large and small airways, during 24 months' ETI therapy in pwCF aged ≥6 years. Notable improvements, particularly in small airways, were observed even in children and adolescents with normal ppFEV<sub>1</sub> before ETI. These findings underscore the importance of early ETI therapy initiation to prevent irreversible lung damage.
Authors
- Matthias Kappler (ORCID: https://orcid.org/0000-0003-3889-9552)
- Katharina Schütz (ORCID: https://orcid.org/0000-0003-2680-3541)
- Folke Brinkmann (ORCID: https://orcid.org/0000-0002-4307-4232)
- Sivagurunathan Sutharsan (ORCID: https://orcid.org/0000-0003-4069-4900)
- Helmut Ellemunter (ORCID: https://orcid.org/0000-0003-0402-1207)
- Lutz Naehrlich (ORCID: https://orcid.org/0000-0002-7146-6997)
- Sarah Sieber
- Stefanie Dillenhoefer
- Manuel Burkhart
Institutions
- Statistisches Bundesamt (DE)
- Innsbruck Medical University (AT)
- Universität Innsbruck (AT)
- Justus-Liebig-Universität Gießen (DE)
- Medizinische Hochschule Hannover (DE)
- Ruhrlandklinik (DE)
- The Cystic Fibrosis Institute (DE)
- Universities of Giessen and Marburg Lung Center (DE)
- St. Josef-Hospital (DE)
- Statistical Service (CY)
- University of Duisburg-Essen (DE)
- Ludwig-Maximilians-Universität München (DE)
- Ruhr University Bochum (DE)
- University of Lübeck (DE)
Publication Details
- Journal
- ERJ Open Research
- Published
- 2025-10-09
- DOI
- https://doi.org/10.1183/23120541.00813-2025
- Primary Topic
- Breastfeeding Practices and Influences
- Type
- article
- Field-Weighted Citation Impact
- 0.00