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CF Fungal Disease in the Age of CFTR Modulators

Amelia Bercusson1, George Jarvis2, Anand Shah3,4

  • 1Cystic Fibrosis Unit, University Hospital Southampton NHS Foundation Trust, Southampton, UK.

Mycopathologia
|April 4, 2021
PubMed

Insights

Novel CFTR modulators are changing cystic fibrosis (CF) treatment, impacting fungal lung disease and antifungal immunity. This review explores their effects on Aspergillus fumigatus infections and drug interactions in CF patients.

Area of Science:

  • Pulmonology
  • Mycology
  • Pharmacology

Background:

  • Fungi, particularly Aspergillus fumigatus, significantly contribute to lung disease progression in cystic fibrosis (CF).
  • Novel Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators are transforming CF disease management and patient outcomes.
  • The interplay between CFTR modulators and fungal lung infections in CF requires thorough investigation.

Purpose of the Study:

  • To review the impact of novel CFTR modulators on fungal lung disease in cystic fibrosis.
  • To examine how CFTR modulators influence antifungal innate immunity.
  • To assess the effects of specific modulators like Ivacaftor on fungal infections in CF patients with gating mutations.

Main Methods:

  • Literature review focusing on studies of CFTR modulators and fungal infections in cystic fibrosis.
  • Analysis of existing data on the effects of CFTR modulators on immune responses to fungi.
  • Examination of reported drug-drug interactions between CFTR modulators and antifungal agents.

Main Results:

  • CFTR modulators may alter the host's immune defenses against fungal pathogens.
  • Ivacaftor's specific impact on fungal disease in individuals with gating mutations is a key area of interest.
  • Concurrent use of azole antifungals with CFTR modulators presents a growing challenge due to drug-drug interactions.

Conclusions:

  • Understanding the impact of CFTR modulators on fungal lung disease is crucial for optimizing CF care.
  • Further research is needed to address key unknowns regarding CFTR modulators, fungal immunity, and drug interactions in CF.
  • Personalized management strategies may be required to balance the benefits of CFTR modulators with the risks of fungal infections and drug interactions.
Keywords:
AntifungalAspergillus fumigatusCystic fibrosisFungi

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