Figure 2.

Potential effects of CFTR modulators on inflammation and host responses in cystic fibrosis (CF). (a) In CF airways, depleted airway surface liquid (ASL) and poor mucociliary clearance (MCC) leads to mucus adherence to exposed epithelial surfaces. As a result, PWCF have increased susceptibility to, and reduced clearance of, pathogens as well as dysregulated host responses such as impaired neutrophil and macrophage phagocytosis and delayed neutrophil apoptosis. (b) With CFTR modulator therapy, restoration of the ASL barrier may be associated with improved MCC and epithelial integrity, increased microbial killing, and clearance due to the restoration of host defence mechanisms, including leukocyte phagocytic function.