What is the recommended dosage and duration of prednisone (corticosteroid) for patients with cystic fibrosis (CF)?

Prednisone Use in Cystic Fibrosis: Recommendations for Dosage and Duration

The Cystic Fibrosis Foundation recommends against the chronic use of oral corticosteroids in patients with cystic fibrosis due to negative net clinical benefit, despite some evidence of improved lung function. 1

Evidence on Prednisone Use in CF

Efficacy

• Alternate-day prednisone at 1 mg/kg has shown some benefit in slowing lung function decline in CF patients, particularly those colonized with Pseudomonas aeruginosa 2
• Studies demonstrated that patients receiving 1 mg/kg alternate-day prednisone maintained better FEV1 over 48 months compared to placebo (-2% vs -6% decline) 3, 4
• Short-term use (5 days) of prednisone at 2 mg/kg/day (maximum 60 mg) during acute pulmonary exacerbations showed a trend toward improvement in lung function, though results were not statistically significant 5

Adverse Effects

• Significant adverse effects have been documented with long-term prednisone use in CF patients, including 1:
  • Linear growth retardation (observed as early as 6 months with 2 mg/kg alternate-day dosing and from 24 months with 1 mg/kg alternate-day dosing)
  • Abnormalities in glucose metabolism
  • Cataracts
  • Increased colonization with Pseudomonas aeruginosa

Current Recommendations

Long-term Use

• The Cystic Fibrosis Foundation explicitly recommends against the chronic use of oral corticosteroids in CF patients aged 6-18 years without asthma or allergic bronchopulmonary aspergillosis (ABPA) 1
• This recommendation is graded as "Level of evidence, good; net benefit, negative; grade of recommendation, D" 1

Short-term Use

• For acute pulmonary exacerbations, short-term prednisone (2 mg/kg/day up to 60 mg for 5 days) may be considered, though evidence for significant benefit is limited 5
• When treating comorbid conditions like ABPA in CF patients, standard corticosteroid protocols for those specific conditions should be followed 1

Historical Context

• The largest study on prednisone in CF (Eigen et al.) involved 285 patients aged 6-14 years who received either alternate-day prednisone (1 or 2 mg/kg; maximum 60 mg) or placebo for up to 4 years 2
• This study was terminated early by the data safety monitoring committee due to excessive adverse events in both prednisone groups 1, 2
• Despite some preservation of lung function, the adverse effects outweighed the benefits 2, 3

Clinical Considerations

• If corticosteroids are required for comorbid conditions in CF patients:
  • Use the lowest effective dose for the shortest duration possible 3, 4
  • Monitor closely for adverse effects, particularly glucose abnormalities and growth in pediatric patients 1, 2
  • Consider alternative anti-inflammatory approaches with better safety profiles 1

Common Pitfalls

• Initiating long-term corticosteroids for CF lung disease without considering the significant risk of adverse effects 1, 2
• Failing to distinguish between CF patients who have comorbid conditions requiring corticosteroids (such as ABPA or asthma) and those who do not 1
• Not monitoring for steroid-related complications, particularly glucose metabolism abnormalities and growth retardation in children 2, 3