Inflammation and cystic fibrosis pulmonary disease

Inflammation plays a primary role in the pathogenesis of cystic fibrosis (CF)-related lung disease. Controlling the inflammatory process with antiinflammatory therapy may slow the progression of pulmonary disease and thereby decrease morbidity. Despite potential benefits of antiinflammatory therapy, both the decision to treat and selection of the most appropriate therapeutic agent are controversial. Although oral corticosteroids are associated with reduced progression of pulmonary disease, the risk of clinically significant adverse effects limits long-term therapy. Clinical studies with inhaled corticosteroids failed to report positive effects on reducing airway inflammation. Based on available clinical data, routine therapy with these agents should be limited to patients with asthma or steroid-responsive wheezing. High-dosage ibuprofen has a beneficial effect on reducing the annual rate of decline in pulmonary function in patients with mild lung disease. Whereas initial results are encouraging, they do not support routine ibuprofen therapy in all patients with CE However, as advocated by the Cystic Fibrosis Foundation, high-dosage ibuprofen may be considered in children 5-12 years of age with a baseline forced expiratory volume of 60% predicted or greater.