Reduction of systemic inflammation by elexacaftor/tezacaftor/ivacaftor correlates with lung function improvement in cystic fibrosis
Our results demonstrate that ETI exerts rapid and sustained effects on systemic inflammation associated with lung function improvements in children, adolescents and adults with cystic fibrosis in a real-world, post-approval setting. However, our data also show that individual markers of systemic inflammation remain at levels above those of healthy controls, particularly in certain subgroups, suggesting persistence or resurgence of residual systemic inflammation.
Authors
- Katharina Schütz (ORCID: https://orcid.org/0000-0003-2680-3541)
- Folke Brinkmann (ORCID: https://orcid.org/0000-0002-4307-4232)
- Stephanie A. Thee (ORCID: https://orcid.org/0000-0001-9090-7869)
- Stephanie Hirtz
- Ruth Grychtol (ORCID: https://orcid.org/0000-0002-8084-104X)
- Julia Kontsendorn
- Tatjana Uselmann
- Jobst Roehmel (ORCID: https://orcid.org/0000-0002-1535-8852)
- Olaf Sommerburg (ORCID: https://orcid.org/0000-0002-3941-8944)
- Anna‐Maria Dittrich (ORCID: https://orcid.org/0000-0002-9582-1025)
- Burkhard Tümmler (ORCID: https://orcid.org/0000-0002-2566-8758)
- Felix C. Ringshausen (ORCID: https://orcid.org/0000-0001-9084-4968)
- Marcus Alexander Mall (ORCID: https://orcid.org/0000-0002-4057-2199)
- Johannes Schwabe (ORCID: https://orcid.org/0000-0002-0916-3098)
- Lutz Naehrlich (ORCID: https://orcid.org/0000-0002-7146-6997)
- Bianca Schaub (ORCID: https://orcid.org/0000-0003-1652-8873)
- Iris Kühbandner
- Jan-Niklas Falke
- Simon Y. Graeber (ORCID: https://orcid.org/0000-0002-1666-8924)
- Claudia Kessemeier
- Jaehi Chung
- Zulfiya Syunyaeva
- Markus Weckmann (ORCID: https://orcid.org/0000-0001-5342-979X)
- Claudia Rückes-Nilges
- Olga Halle (ORCID: https://orcid.org/0000-0003-3814-7844)
- Silke van Koningsbruggen‐Rietschel (ORCID: https://orcid.org/0000-0002-2222-4238)
- Gesine Hansen (ORCID: https://orcid.org/0000-0001-5185-9454)
- Stefanie Widder (ORCID: https://orcid.org/0000-0003-0733-5666)
- Mirjam Stahl (ORCID: https://orcid.org/0000-0001-6941-8315)
- Mustafa Abdo (ORCID: https://orcid.org/0000-0002-3300-1398)
- Julia Duerr (ORCID: https://orcid.org/0000-0002-4862-9487)
- Sophia T. Pallenberg (ORCID: https://orcid.org/0000-0002-9948-7589)
- Stefanie Weber (ORCID: https://orcid.org/0000-0002-9498-2337)
- Sandra Barth
- Rebecca Dalferth
- Azadeh Bagheri-Pothoff
Institutions
- American Institute of Biological Sciences (US)
- Justus-Liebig-Universität Gießen (DE)
- Heidelberg University (DE)
- University Hospital Heidelberg (DE)
- Medizinische Hochschule Hannover (DE)
- Humboldt-Universität zu Berlin (DE)
- Equilibrium Research (GB)
- Research Center Borstel - Leibniz Lung Center (DE)
- German Center for Lung Research (DE)
- Center for Health and Gender Equity (US)
- Universities of Giessen and Marburg Lung Center (DE)
- Berlin Institute of Health at Charité - Universitätsmedizin Berlin (DE)
- University Hospital Cologne (DE)
- Freie Universität Berlin (DE)
- Medical University of Vienna (AT)
- Charité - Universitätsmedizin Berlin (DE)
- Ludwig-Maximilians-Universität München (DE)
- Deutsche Forschungsgemeinschaft (DE)
- University of Lübeck (DE)
Publication Details
- Journal
- European Respiratory Journal
- Published
- 2025-09-18
- DOI
- https://doi.org/10.1183/13993003.00150-2025
- Citations
- 4
- Primary Topic
- Cystic Fibrosis Research Advances
- Type
- article
- Field-Weighted Citation Impact
- 6.07