BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to Aspergillus fumigatus contributing to cystic fibrosis (CF) lung disease. OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.
- Gothe, F.
- Schmautz, A.
- Hausler, K.
- Tran, N. B.
- Kappler, M.
- Griese, M.
Keywords
- Abpa
- Aspergillus fumigatus
- Cystic fibrosis
- IgE
- Itraconazole
- Lung function
- Pseudomonas aeruginosa
- Tdm
