Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis.

BACKGROUND

Recent studies demonstrated that the triple combination cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) improves lung function and reduces pulmonary exacerbations in cystic fibrosis (CF) patients with at least one

METHODS

In this prospective observational study, we assessed sputum rheology, the microbiome, inflammation markers and proteome before and 1, 3 and 12 months after initiation of ETI.

RESULTS

In total, 79 patients with CF and at least one

CONCLUSIONS

Our data demonstrate that restoration of CFTR function by ETI improves sputum viscoelastic properties, chronic airway infection and inflammation in CF patients with at least one

Informations de copyright

Copyright ©The authors 2023. For reproduction rights and permissions contact [e-mail].

Déclaration de conflit d'intérêts

Conflict of interest: J. Röhmel reports payment for presentations at educational events from Vertex Pharmaceuticals, outside the submitted work. D. Lauster has received funding from Deutsche Forschungsgemeinschaft for work related to this manuscript. M. Stahl reports funding from Deutsche Forschungsgemeinschaft related to this manuscript, and grants from Vertex Pharmaceuticals and Mukoviszidose e.V. (German CF Foundation) outside the submitted work, payment for work on an advisory board from Vertex Pharmaceuticals, and is elected, unpaid secretary of the group on paediatric CF of Assembly 7 of the ERS. P. Mertins received funding from Deutsche Forschungsgemeinschaft and the German Federal Ministry of Education and Research related to this work. S.Y. Graeber reports grant support from Mukoviszidose e.V. (German CF Foundation) and Vertex Pharmaceuticals Incorporated outside the submitted work, with payments made to the author's institution. Chiesi GmbH and Vertex Pharmaceuticals Incorporated have provided personal fees for presentations and advisory boards. M.A. Mall declares that he has received funding from Deutsche Forschungsgemeinschaft and German Ministry for Education and Research for work related to this manuscript. In the past 36 months, he has received grants from Vertex Pharmaceuticals, personal fees for consultancy from Boehringer Ingelheim, Arrowhead Pharmaceuticals, Vertex Pharmaceuticals, Santhera, Sterna Biologicals, Enterprise Therapeutics, Antabio and Abbvie, lecture fees from Boehringer Ingelheim, Arrowhead Pharmaceuticals and Vertex Pharmaceuticals, travel reimbursement from Boehringer Ingelheim and Vertex Pharmaceuticals, and personal fees for participation in advisory boards from Boehringer Ingelheim, Arrowhead Pharmaceuticals, Vertex Pharmaceuticals, Santhera, Enterprise Therapeutics, Antabio, Kither Biotech, Abbvie and Pari. Additionally, he served as a member of the Board and Vice-President of the European Cystic Fibrosis Society from 2014 to 2020. All other authors have nothing to disclose.