Airway Epithelium Dysfunction in Cystic Fibrosis and COPD

Figure 1

Similarities and differences between cystic fibrosis and COPD. Cystic fibrosis (CF) is a genetic disease caused by mutations in the CFTR gene, while COPD is a multifactorial-acquired disease of later onset predominantly associated with cigarette smoke exposure. Although the diseases are different in many aspects, they also share key phenotypical features. Recent evidence suggests that cigarette smoke induces an acquired CFTR dysfunction in COPD and that both CF and COPD have corresponding molecular phenotypes of CFTR dysfunction resulting in airway surface liquid (ASL) dehydration, mucostasis, bacterial colonization, chronic inflammation, and tissue damage. Airflow obstruction, recurrent infective exacerbations, and a progressive decline in lung function characterize both conditions.