Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy.

Autor:	Milczewska J; Cystic Fibrosis Department, Institute of Mother and Child, Warsaw, Poland.; Cystic Fibrosis Centre, Pediatric Hospital, Dziekanow Lesny, Poland.; Joint first authors., Syunyaeva Z; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany.; Joint first authors., Żabińska-Jaroń A; Cystic Fibrosis Centre, Pediatric Hospital, Dziekanow Lesny, Poland., Sands D; Cystic Fibrosis Department, Institute of Mother and Child, Warsaw, Poland.; Cystic Fibrosis Centre, Pediatric Hospital, Dziekanow Lesny, Poland., Thee S; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany stephanie.thee@charite.de.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.
Jazyk:	angličtina
Zdroj:	European respiratory review : an official journal of the European Respiratory Society [Eur Respir Rev] 2024 Dec 04; Vol. 33 (174). Date of Electronic Publication: 2024 Dec 04 (Print Publication: 2024).
DOI:	10.1183/16000617.0068-2024
Abstrakt:	The advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy, especially the triple therapy combining the drugs elexacaftor, tezacaftor, ivacaftor (ETI), has significantly changed the course of the disease in people with cystic fibrosis (pwCF). ETI, which is approved for the majority (80-90%) of pwCF, partially restores CFTR channel function, resulting in improved mucociliary clearance and, consequently, improved lung function, respiratory symptoms and pulmonary exacerbations. The bacterial burden of classical CF pathogens such as Pseudomonas aeruginosa and Staphylococcus aureus is reduced without reaching eradication in the majority of infected patients. Limited data is available on less common or emerging bacterial pathogens. ETI has a positive effect on the lung microbiome but does not fully restore it to a healthy state. Due to the significant reduction in sputum production under ETI, respiratory samples such as deep-throat swabs are commonly taken, despite their inadequate representation of lower respiratory tract pathogens. Currently, there are still unanswered questions related to this new therapy, such as the clinical impact of infection with cystic fibrosis (CF) pathogens, the value of molecular diagnostic tests, the durability of the effects on respiratory infection and the role of fungal and viral infections. This article reviews the changes in bacterial lung infections and the microbiome in CF to provide evidence for the use of antibiotics in the era of ETI. Competing Interests: Conflict of interest: J. Milczewska reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Vertex Pharmaceuticals (Poland) Sp. z o.o. Z. Syunyaeva reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Vertex Pharmaceuticals, and support for attending meetings from Vertex Pharmaceuticals. A. Żabińska-Jaroń has nothing to disclose. D. Sands reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Vertex Pharmaceuticals and Pfizer, support for attending meetings from Vertex Pharmaceuticals, participation on a data safety monitoring board or advisory board with Vertex Pharmaceuticals, and leadership roles with ECFS (Board member) and Polish CF Society (President). S. Thee reports payment or honoraria for lectures, presentations, manuscript writing or educational events from PARI Medical Holding, Vertex Pharmaceuticals and Viatris, support for attending meetings from Vertex Pharmaceuticals and Viatris, and leadership roles with ECFS (THCF working group and pulmonary exacerbation group) and ERS (CRC AMR-lung). (Copyright ©The authors 2024.)
Databáze:	MEDLINE
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