Milczewska Justyna, Syunyaeva Zulfiya, Żabińska-Jaroń Aleksandra, Sands Dorota, Thee Stephanie
Cystic Fibrosis Department, Institute of Mother and Child, Warsaw, Poland.
Cystic Fibrosis Centre, Pediatric Hospital, Dziekanow Lesny, Poland.
Eur Respir Rev. 2024 Dec 4;33(174). doi: 10.1183/16000617.0068-2024. Print 2024 Oct.
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 and 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.
囊性纤维化跨膜传导调节因子（CFTR）调节剂疗法的出现，尤其是艾列卡福托、替扎卡福托、依伐卡托（ETI）三联药物疗法，显著改变了囊性纤维化患者（pwCF）的疾病进程。ETI已获批用于大多数（80%-90%）的pwCF患者，可部分恢复CFTR通道功能，从而改善黏液纤毛清除功能，进而改善肺功能、缓解呼吸道症状并减少肺部急性加重。在大多数感染患者中，铜绿假单胞菌和金黄色葡萄球菌等典型CF病原体的细菌载量会降低，但不会被根除。关于不太常见或新出现的细菌病原体的数据有限。ETI对肺部微生物群有积极影响，但不能使其完全恢复到健康状态。由于在ETI治疗下痰液分泌显著减少，尽管深喉拭子等呼吸道样本不能充分代表下呼吸道病原体，但仍常被采集。目前，关于这种新疗法仍有一些未解决的问题，如感染囊性纤维化（CF）病原体的临床影响、分子诊断测试的价值、对呼吸道感染影响的持续性以及真菌和病毒感染的作用。本文综述了CF患者肺部细菌感染和微生物群的变化，为ETI时代抗生素的使用提供依据。
