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Evidence summaries

Oral Non-Steroidal Anti-Inflammatory Drugs for Lung Disease in Cystic Fibrosis

High-dose ibuprofen appears to slow the progression of lung disease in people with cystic fibrosis, but there are limited long-term safety data. Level of evidence: "B"

A Cochrane review [Abstract] 1 included 4 studies with a total of 287 subjects aged 5 to 39 years. Maximum follow up time was 4 years. 3 studies compared ibuprofen to placebo, 1 study compared piroxicam to placebo. Combined data from the two largest ibuprofen trials (n=226) showed a significantly lower annual rate of decline for lung function, % predicted forced expiratory volume in one second (FEV1) (MD 1.32, 95% CI 0.21 to 2.42); FVC (MD 1.27, 95% CI 0.26 to 2.28); and forced expiratory flow (25-75%)(MD 1.80, 95% CI 0.15 to 3.45). The post-hoc analysis of data from two trials split by age showed a statistically significant slower rate of annual decline of % predicted FEV1 and FVC in the ibuprofen group in younger children (MD 1.41%, 95% CI 0.03 to 2.80 and MD 1.32%, 95% CI 0.04 to 2.60, respectively). 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.

Comment: The quality of evidence is downgraded by imprecise results (few patients).

    References

    • Lands LC, Stanojevic S. Oral non-steroidal anti-inflammatory drug therapy for lung disease in cystic fibrosis. Cochrane Database Syst Rev 2016;(4):CD001505.[PubMed].

Primary/Secondary Keywords