Airway Inflammation and Host Responses in the Era of CFTR Modulators

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Abstract

The arrival of cystic fibrosis transmembrane conductance regulator (CFTR) modulators as a new class of treatment for cystic fibrosis (CF) in 2012 represented a pivotal advance in disease management, as these small molecules directly target the upstream underlying protein defect. Further advancements in the development and scope of these genotype-specific therapies have been transformative for an increasing number of people with CF (PWCF). Despite clear improvements in CFTR function and clinical endpoints such as lung function, body mass index (BMI), and frequency of pulmonary exacerbations, current evidence suggests that CFTR modulators do not prevent continued decline in lung function, halt disease progression, or ameliorate pathogenic organisms in those with established lung disease. Furthermore, it remains unknown whether their restorative effects extend to dysfunctional CFTR expressed in phagocytes and other immune cells, which could modulate airway inflammation. In this review, we explore the effects of CFTR modulators on airway inflammation, infection, and their influence on the impaired pulmonary host defences associated with CF lung disease. We also consider the role of inflammation-directed therapies in light of the widespread clinical use of CFTR modulators and identify key areas for future research.

Original languageEnglish
JournalInternational journal of molecular sciences
Volume21
Issue number17
DOIs
Publication statusPublished - 02 Sep 2020

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  • Student Theses

    Cathepsin S as a mediator of acute lung inflammation

    Author: McKelvey, M., Dec 2020

    Supervisor: Taggart, C. (Supervisor), Weldon, S. (Supervisor) & McAuley, D. (Supervisor)

    Student thesis: Doctoral ThesisDoctor of Philosophy

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