Elexacaftor – Tezacaftor – Ivacaftor treatment improves systemic infection parameters and Pseudomonas aeruginosa colonization rate in patients with cystic fibrosis a monocentric observational study

Schnell, Alexander; Hober, Hannah; Kaiser, Natalie; Ruppel, Renate; Geppert, Annika; Tremel, Christina; Sobel, Julia; Plattner, Erika; Woelfle, Joachim; Hoerning, André

doi:10.1016/j.heliyon.2023.e15756

Cited by 10 publications

(2 citation statements)

References 33 publications

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“…Highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy (HEMT), including elexacaftor/tezacaftor/ivacaftor (ETI), is now approved for more than 90% of adults with the genetic disorder cystic fibrosis. ETI dramatically reduces upper and lower respiratory symptoms and improves outcomes, yet it is unclear whether established bacterial infections in the airways of people with CF (pwCF) on ETI are eradicated ( 1 – 4 ). One challenge of this new era of HEMT is that many pwCF fail to produce sputum that is needed to monitor airway infections by opportunistic pathogens such as Pseudomonas aeruginosa .…”

Section: Observationmentioning

confidence: 99%

Persistence and evolution of Pseudomonas aeruginosa following initiation of highly effective modulator therapy in cystic fibrosis

Armbruster,

Hilliam,

Zemke

et al. 2024

mBio

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Today, more than 90% of people with cystic fibrosis (pwCF) are eligible for the highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy called elexacaftor/tezacaftor/ivacaftor (ETI) and its use is widespread. Given the drastic respiratory symptom improvement experienced by many post-ETI, clinical studies are already underway to reduce the number of respiratory therapies, including antibiotic regimens, that pwCF historically relied on to combat lung disease progression. Early studies suggest that bacterial burden in the lungs is reduced post-ETI, yet it is unknown how chronic Pseudomonas aeruginosa populations are impacted by ETI. We found that pwCF remain infected throughout their upper and lower respiratory tract with their same strain of P. aeruginosa post-ETI, and these strains continue to evolve in response to the newly CFTR-corrected airway. Our work underscores the continued importance of CF airway microbiology in the new era of highly effective CFTR modulator therapy. IMPORTANCE The highly effective cystic fibrosis transmembrane conductance regulator modulator therapy Elexakaftor/Tezacaftor/Ivacaftor (ETI) has changed cystic fibrosis (CF) disease for many people with cystic fibrosis. While respiratory symptoms are improved by ETI, we found that people with CF remain infected with Pseudomonas aeruginosa . How these persistent and evolving bacterial populations will impact the clinical manifestations of CF in the coming years remains to be seen, but the role and potentially changing face of infection in CF should not be discounted in the era of highly effective modulator therapy.

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Section: Observationmentioning

confidence: 99%

Persistence and evolution of Pseudomonas aeruginosa following initiation of highly effective modulator therapy in cystic fibrosis

Armbruster,

Hilliam,

Zemke

et al. 2024

mBio

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“…Yet, current CFTR modulators do not achieve WT function of CFTR (11) and current CFTR modulator treatment is largely addressing pwCF with irreversible lung damage due to longstanding disease. So far, data suggest that the dysbiosis observed in CF is only partially addressed even by ELX/TEZ/IVA (19)(20)(21)(22), the most effective and broadly applicable CFTR modulator developed so far. Until CFTR modulators show even higher improvements of CFTR function and pwCF are treated before the onset of irreversible, structural lung damage, symptom-directed anti-infective therapy, for instance in the event of viral-induced exacerbations and/or loss of lung function will most likely remain a necessary companion of the causal approach offered by modulator therapies.…”

Section: To the Editormentioning

confidence: 99%

Optimal treatment of the underlying aetiology is the most effective antimicrobial stewardship for chronic respiratory disease: a lesson learned from cystic fibrosis

Rademacher,

Martin,

Theloe

et al. 2023

ERJ Open Res

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Extract The increasing occurrence of antimicrobial resistance in bacterial infections has emerged as one of the biggest threats to global health. Antimicrobial stewardship (AMS) programmes have grown rapidly [1, 2] over recent decades due to increasing evidence of their effect on the quality of medical care, by reducing adverse outcomes, bacterial resistance, drug-related toxicity and secondary infections [3]. Furthermore, they play a crucial role in reducing community resistance rates. National surveillance programmes of antimicrobial resistance providing yearly reports and interactive data platforms are well developed in most high-income countries. In low- and middle-income countries, major gaps exist, ranging from limited expertise, infrastructure, investment and access to antimicrobials, and lack of microbiological diagnostic capabilities [4].

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Use of elexacaftor/tezacaftor/ivacaftor leads to changes in detection frequencies of Staphylococcus aureus and Pseudomonas aeruginosa dependent on age and lung function in people with cystic fibrosis

Dittrich,

Sieber,

Naehrlich

et al. 2024

International Journal of Infectious Diseases

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Elexacaftor – Tezacaftor – Ivacaftor treatment improves systemic infection parameters and Pseudomonas aeruginosa colonization rate in patients with cystic fibrosis a monocentric observational study

Cited by 10 publications

References 33 publications

Persistence and evolution of Pseudomonas aeruginosa following initiation of highly effective modulator therapy in cystic fibrosis

Persistence and evolution of Pseudomonas aeruginosa following initiation of highly effective modulator therapy in cystic fibrosis

Optimal treatment of the underlying aetiology is the most effective antimicrobial stewardship for chronic respiratory disease: a lesson learned from cystic fibrosis

Use of elexacaftor/tezacaftor/ivacaftor leads to changes in detection frequencies of Staphylococcus aureus and Pseudomonas aeruginosa dependent on age and lung function in people with cystic fibrosis

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