Patients with cystic fibrosis (CF) experience declining pulmonary function related to chronic
airway inflammation, which results from epithelial and immune cell secretion of
proinflammatory mediators that promote neutrophil influx into the airways. This inflammatory
response may be disproportionate to the inciting infectious stimulus, resulting in an overly
exuberant influx of neutrophils. The neutrophils release proteases, including neutrophil
elastase, that eventually overwhelm the antiprotease capacity of the lung and cleave …