Background Progressive lung damage causes the majority of deaths in cystic fibrosis ( CF). Non-steroidal anti-inflammatory drugs may prevent progressive pulmonary deterioration and morbidity in CF. Objectives To assess the effectiveness of treatment with non-steroidal anti-inflammatory agents in CF. Search strategy We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register comprising references identified from comprehensive electronic database searches, hand searches of relevant journals and abstract books of conference proceedings. We also contacted pharmaceutical companies manufacturing non-steroidal anti-inflammatory drugs. Most recent search of the Group's Trials Register: October 2006. Selection criteria Randomized or quasi-randomized controlled trials, published and unpublished, comparing oral non-steroidal anti-inflammatory drugs, at any dose for at least two months, to placebo in people with CF. Data collection and analysis Two authors independently assessed trials for the review. Main results The searches identified six trials, of which four, including 287 participants aged five to 39 years with a maximum follow up of four years, were eligible for inclusion in the review. Two trials reporting effectiveness of ibuprofen in people with mild lung disease were from the same center and included some of the same participants. A third assessed piroxicam in participants with more severe impairment of respiratory function and the Trans-Canada trial compared ibuprofen to placebo for a period of two years. Three of the trials in this review were deemed to have good or adequate methodological quality, but variation in outcomes reported and their summary measures precluded calculation of pooled treatment estimates. Authors considered objective measures of lung function, nutritional status, radiological assessment of pulmonary involvement, intravenous antibiotic usage, hospital admissions, survival, frequency of all adverse effects and compliance with therapy. The addition of data from the Canadian trial showed evidence of a moderate absolute annual decline in per cent predicted forced expiratory volume in one second and forced vital capacity in the placebo group than in the ibuprofen group. In one trial, long-term use of high-dose ibuprofen was associated with reduced intravenous antibiotic usage, improved nutritional and radiological pulmonary status. No major adverse effects were reported, but the power of the trials to identify clinically important differences in the incidence of adverse effects was low. Authors' conclusions High-dose ibuprofen can slow the progression of lung disease in people with CF, especially in children, and this suggests that strategies to modulate lung inflammation can be beneficial for people with CF.
