When allergic bronchopulmonary aspergillosis (ABPA) occurs in a patient with cystic fibrosis (CF), the clinical approach must account for the specific challenges that CF introduces alongside the standard ABPA treatment framework.
This protocol is designed for ABPA arising in the context of cystic fibrosis. The core treatment principles applied mirror those established for ABPA in asthma, but require careful adaptation to the physiological and pharmacological considerations specific to CF.
Management involves anti-inflammatory therapy, with antifungal agents used in a complementary role — the selection between available options is guided by the need to balance efficacy against the tolerability concerns that are particularly relevant in the CF population.
DOI: 10.1183/13993003.00061-2024
We recommend diagnosing and treating CF-ABPA using the same treatment tenets outlined for ABPA in asthma; however, exercising due caution for issues specific to CF (LoC: 100%).
Glucocorticoids or azoles are the preferred initial agents.
Monthly doses of intravenous methylprednisolone therapy alone or with azoles have been used to limit the toxicity associated with daily glucocorticoids.
A 3-week course of oral corticosteroids combined with oral itraconazole for 12 months effectively treated CF-ABPA.
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