Identifying the Origins of Cystic Fibrosis Lung Disease

Although the median survival age has improved steadily during the past half century, cystic fibrosis is still a life-shortening disease, affecting 30,000 children and adults in the United States. The airways of patients with cystic fibrosis are susceptible to infection, which induces an intense inflammatory response even in those with only modest pulmonary disease.1 Although they are often asymptomatic, infants with cystic fibrosis have neutrophilic inflammation in their airways, which is both protective and destructive. Neutrophil proteases, including elastase, are critical for killing engulfed bacteria, but these proteases escape from neutrophils during phagocytosis and in death and subsequently overwhelm antiprotease . . .