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Evidence summaries

Long-Term Oral Steroids for Cystic Fibrosis

Oral steroids at prednisolone equivalent dose of 1-2 mg/kg alternate days may slow the progression of lung disease in cystic fibrosis but cause cataracts and impair growth. Level of evidence: "C"

A Cochrane review [Abstract] 1 on long-term (over 30 days) use of oral steroids for cystic fibrosis included 3 studies with a total of 354 subjects. Follow up was 4 years in 2 studies, and 12 weeks in 1 study. Meta-analyses were not possible.

In one study, oral corticosteroids at a prednisolone equivalent dose of 1 mg/kg alternate days slowed the progression of lung disease; at 24 months from commencement, 70.4% of patients had an increase in % predicted FVC compared to 41.6% of patients treated with placebo; at two and four years, % predicted FEV1 in the 1 mg/kg group changed significantly more than in the placebo group (P < 0.02). During the first two years, the 2 mg/kg group was not significantly different from the placebo group. Linear growth retardation was observed from six months in the 2 mg/kg alternate days prednisolone group and from 24 months in the 1 mg/kg alternate days prednisolone group.

Adverse events terminated one four-year study early. Year 10 follow up showed catch-up growth started two years after treatment ceased. Alternate-day treatment with oral corticosteroids may have impaired growth until adulthood in boys.

Clinical comment: The authors conclude that current evidence suggests that oral corticosteroids at a prednisolone equivalent dose of 2 mg/kg on alternate days is effective but should not be used due to the high risk of occurrence of important side effects. A dose of 1 mg/kg on alternate days might be considered for up to 24 months but close attention to the occurrence of adverse effects is warranted.

Comment: The quality of evidence is downgraded by study limitations (unclear allocation concealment) and by imprecise results (few patients).

    References

    • Cheng K, Ashby D, Smyth RL. Oral steroids for long-term use in cystic fibrosis. Cochrane Database Syst Rev 2015;(12):CD000407. [PubMed].

Primary/Secondary Keywords