Purpose of review: Ibuprofen was first advocated as a chronic therapy for cystic fibrosis lung disease in 1995, following a favorable report of a 4-year controlled trial. However, clinical use has been limited primarily by the concern about adverse effects. Additional clinical studies were advocated to better assess the risk-benefit profile. The results of several studies have been published within the last couple of years.
Recent findings: Results from a 2-year multicenter trial were consistent with the 4-year trial in demonstrating a beneficial effect of ibuprofen on lung function in children with mild to moderate lung disease. The drug also showed a favorable safety profile. Results from an analysis of observational data from the Cystic Fibrosis Foundation Patient Registry collected over 7 years revealed that 'real world' clinical use of ibuprofen was also associated with a beneficial effect. The occurrence of gastrointestinal bleeding was higher in those treated with ibuprofen, but the incidence was very low.
Summary: Ibuprofen has now been shown in two long-term clinical trials to slow disease progression, with real-world clinical use supporting its effectiveness. Although the therapy is not without adverse effects, the benefits appear to outweigh the risks.