Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy
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 fo...
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| Format: | Article |
| Language: | English |
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European Respiratory Society
2024-12-01
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| Series: | European Respiratory Review |
| Online Access: | http://err.ersjournals.com/content/33/174/240068.full |
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| author | Justyna Milczewska Zulfiya Syunyaeva Aleksandra Żabińska-Jaroń Dorota Sands Stephanie Thee |
| author_facet | Justyna Milczewska Zulfiya Syunyaeva Aleksandra Żabińska-Jaroń Dorota Sands Stephanie Thee |
| author_sort | Justyna Milczewska |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-f7d0529554fc4dc9aa5c3da5dbb8b1e6 |
| institution | DOAJ |
| issn | 0905-9180 1600-0617 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | European Respiratory Review |
| spelling | doaj-art-f7d0529554fc4dc9aa5c3da5dbb8b1e62025-08-20T02:57:45ZengEuropean Respiratory SocietyEuropean Respiratory Review0905-91801600-06172024-12-013317410.1183/16000617.0068-20240068-2024Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapyJustyna Milczewska0Zulfiya Syunyaeva1Aleksandra Żabińska-Jaroń2Dorota Sands3Stephanie Thee4 Cystic Fibrosis Department, Institute of Mother and Child, Warsaw, Poland 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 Cystic Fibrosis Centre, Pediatric Hospital, Dziekanow Lesny, Poland Cystic Fibrosis Department, Institute of Mother and Child, Warsaw, Poland 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 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.http://err.ersjournals.com/content/33/174/240068.full |
| spellingShingle | Justyna Milczewska Zulfiya Syunyaeva Aleksandra Żabińska-Jaroń Dorota Sands Stephanie Thee Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy European Respiratory Review |
| title | Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy |
| title_full | Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy |
| title_fullStr | Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy |
| title_full_unstemmed | Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy |
| title_short | Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy |
| title_sort | changing profile of bacterial infection and microbiome in cystic fibrosis when to use antibiotics in the era of cftr modulator therapy |
| url | http://err.ersjournals.com/content/33/174/240068.full |
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