TY - JOUR T1 - Inflammation and infection in cystic fibrosis - hen or egg? JF - European Respiratory Journal JO - Eur Respir J SP - 4 LP - 5 DO - 10.1183/09031936.01.17100040 VL - 17 IS - 1 AU - N. Høiby Y1 - 2001/01/01 UR - http://erj.ersjournals.com/content/17/1/4.abstract N2 - The noninflammatory defence of the lungs consists of: 1) the primary noninflammatory, nonspecific defence mechanisms, such as the mucociliary escalator, coughing, alveolar macrophages, defensins and surfactant; and 2) the secondary noninflammatory, specific defence mechanisms, such as secretory immunoglobulin A (S-IgA) 1. The action of these defence mechanisms is silent and very effective in normal subjects, and their activity does not give rise to any symptoms. No tissue damage is mediated through these defence mechanisms. Congenital defects of the primary noninflammatory defence mechanisms comprise cystic fibrosis (CF) (mucus) 2 and ciliary dyskinesia syndrome (cilia). These defects lead to secondary acute or chronic bacterial infections and recruitment of the primary inflammatory defence mechanisms of the lungs, such as polymorphonuclear (PMN) leukocytes, and the secondary inflammatory defence mechanisms of the lungs, such as immunoglobulin G (IgG). The activity of the inflammatory defence mechanisms may lead to successful killing of the offending pathogens, but in addition may give rise to local and systemic symptoms of inflammation such as fever, tissue damage and impaired function. If the … ER -