Secretory leukocyte protease inhibitor in cystic fibrosis.

Cystic fibrosis (CF) is a genetic disorder that leads to a defect in chloride ion transport and results in pancreatic and pulmonary insufficiency. The pulmonary disease is characterized by bacterial colonization and inflammation with excessive levels of neutrophils and neutrophil elastase within the lung. Neutrophil elastase is considered to be one of the major mediators of the pulmonary damage. Secretory leukocyte protease inhibitor (SLPI) is a natural anti-protease found in the upper airways and has been successfully produced by recombinant technology. SLPI is effective in reducing elastase-induced damage in vitro and in vivo and has recently been administered safely as an aeroeol to CF patients with evidence of biochemical efficacy.