Pulmonary manifestation of cystic fibrosis

Pulmonary manifestations of cystic fibrosis (CF) are responsible for almost 90% of the disease-related mortality. The relationship between CFTR dysfun ction and pathophysiology of lung disease is still unclear. The abnormally high absorption of airway liquid depletes the periciliar liquid layer and i mpairs mucociliary clearance as part of the local immune defense. Besides a irway colonisation with pathogens like Staphylococcus aureus, Haemophilus i nfluenzae, Pseudomonas aeruginosa and Burkholderia cepacia peripheral bronc hioles are obstructed by mucous plugs. The course of lung disease is determ ined by marked neutrophilic bronchial inflammation which may be causally re lated to the CFTR defect itself. This leads to bronchiectases, destruction of the lung and finally respiratory insufficiency. Different approaches to symptomatic therapy have been developed which resulted in a dramatic improv ement of life expectancy within the last decades.