CHRONIC endobronchial inflammation and bacterial infection are the main cau
ses of morbidity and mortality in cystic fibrosis (CF), an autosomal recess
ive genetic disorder associated with improper function of chloride channels
. Inflammation in CF lung is greatly amplified after Pseudomonas aeruginosa
infection. In this study the relationship between P. aeruginosa status and
inflammatory markers has been investigated. Seventeen CF children in acute
lung exacerbation were examined. CF patients without P. aeruginosa infecti
on mere characterized by elevated activity of sputum elastase, reduced resp
onse of peripheral blood lymphocytes to PHA and sig significant resistance
to the antiproliferative action of glucocorticoids. These parameters were n
ormalized after antibiotic treatment. The patients with prolonged P. aerugi
nosa infection demonstrated extremely high levels of elastase activity and
elevated amounts of sputum IL-8 and TNF-alpha. Although antibiotic treatmen
t resulted in clinical improvement, it failed to suppress excessive immune
response in the lung. The data indicate that CF patients with prolonged P.
aeruginosa need the modified treatment, which should include immunomodulati
ng drugs and protease inhibitors as well as antibacterial therapy.