Treating Allergic Bronchopulmonary Aspergillosis with Short-Term Prednisone and Itraconazole in Cystic Fibrosis

Abstract

Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to Aspergillus fumigatus contributing to cystic fibrosis (CF) lung disease. To evaluate the combination of oral prednisone for 18 days together with itraconazole therapy for at least 12 months in CF-related ABPA with regard to long-term pulmonary function and side effects. Sixty-five patients with CF treated for ABPA and 127 patients with CF without ABPA serving as matched controls were retrospectively analyzed for a median period of 4.8 years. Serial lung functions were analyzed alongside clinical, microbiological, and laboratory data including itraconazole therapeutic drug monitoring. The used ABPA treatment regimen restored FEV1 values to pre-ABPA levels within 3 months (P < .0001). Long-term FEV1 courses of patients showed no difference when compared with those of ABPA-free controls. Glucocorticoid treatment was not associated with increased CF-related diabetes incidence, growth restriction, or Pseudomonas aeruginosa acquisition. Patients who experienced ABPA relapses displayed lower itraconazole trough levels during the first 3 months of treatment (P < .05). A decreased risk of ABPA recurrence was further associated with P aeruginosa colonization. The proposed treatment scheme for CF-related ABPA is effective in preserving lung function capacity over years in affected individuals without the known glucocorticoid-associated side effects. Itraconazole therapeutic drug monitoring seems useful to prevent disease flares, for which P aeruginosa-negative patients with CF might be particularly susceptible.