Treatment is designed first to control the acute episodes and then to limit the development of chronic lung disease [19
]. Most cases of ABPA require treatment with systemic corticosteroids, and the treatment of choice is prednisone. Steroid therapy rapidly clears the eosinophilic infiltrates and the associated symptoms, although it is less effective at treating mucus impaction. In asthmatic ABPA patients, the usual starting dose is 0.5
mg/kg/day, taken each morning, and this dose is maintained for 2 to 4 weeks while following the patient clinically and checking the chest radiograph for resolution of the acute process. After this induction treatment, the dose of prednisone should be reduced to 0.5
mg/kg given on alternate days. If mucus impaction persists and is associated with atelectasis, bronchoscopy should be performed to confirm the diagnosis and to attempt to remove the mucus plugs. Following resolution of the acute process, the dose of prednisone should be reduced over 1 to 3 months. Chronic treatment with corticosteroids is controversial, especially in adults, because only minorities of patients with ABPA are at risk of chronic lung disease. The relationship between acute episodes and lung damage is unclear, and the precise dose of prednisone is not certain, since acute exacerbations may continue while the patients are on low doses of steroids.
Children with ABPA usually have CF and may need treatment with higher corticosteroid dosing and long-term corticosteroids to prevent progressive lung damage. Stevens et al. [23
] in the Cystic Fibrosis Foundation Consensus Conference report recommended in ABPA CF patients an initial dose of 2
mg/kg/day of prednisone for 1 week. This is then reduced to 1
mg/kg/day for 1 week that was followed by reducing to alternate day dosing. This prednisone dose is then gradual tapered typically. We usually maintain therapy with a dose of 0.5
mg/kg on alternate days for 3 months and then, after 3 months, the dose of prednisone is tapered over a further 3 months while checking the chest radiograph and the serum IgE level for evidence of relapse. Initially, the serum IgE level should be checked at every visit and, if the level increases by twofold or more, the steroid dose should be increased. We recommend that patients are followed with serum IgE levels and chest radiographs every 6 months for the first 1 to 2 years, and then, if the child remains in remission, it should be possible to reduce the frequency of these studies. Unfortunately, this high and prolonged corticosteroid treatment may induce diabetes in these CF patients. Treatment with itraconazole and omalizumab should be considered as adjunctive treatment of ABPA (see below). Furthermore, their use may be helpful in preventing exacerbation of ABPA in CF patients. Monitoring of ABPA in CF patients is done at multiple levels. These include clinical symptoms of dyspnea, cough and wheezing, pulmonary function studies, serum IgE levels, IgE and IgG specific anti-A. fumigatus
antibodies, blood eosinophilia, and blood glucose.
The antifungal agent itraconazole has been used to reduce the doses of steroids that are required [105
]. Initially, there were only open nonrandomized studies that indicated that itraconazole is a useful adjunct to systemic corticosteroid therapy. Two recent randomized controlled trials have also favored intraconazole use. A double-blind, randomized, placebo-controlled trial of itraconazole 200
mg twice daily dose resulted in decreased IgE levels and an increase in pulmonary function and exercise tolerance. Another randomized, controlled trial showed that treatment of stable ABPA in adults with 400
mg/day itraconazole resulted in a significant reduction in sputum eosinophil count, sputum eosinophilic cationic protein levels, serum IgE concentrations, and Aspergillus
-specific IgG. There was also a reduction in episodes of exacerbation requiring treatment with systemic steroids. In the treatment of children with ABPA, we have used a dose of 10
mg/kg/day of itraconazole. Omalizumab, anti-IgE monoclonal antibody, has been used in uncontrolled reports. Anecdotally, it has been effective, but a randomized controlled trial is necessary.
There is no place for immunotherapy in children with ABPA, since it is ineffective and potentially dangerous. Inhaled anti-inflammatory agents, such as cromoglycate and corticosteroids are not generally thought to be effective. The role of inhaled spores in the pathogenesis of ABPA is unclear, but there is a seasonal incidence of ABPA that is probably related to seasonal changes in mold spore counts. Therefore, it is reasonable to advise patients with ABPA to avoid exposure to places with high spore counts, such as damp basements, barns, and compost heaps.