Beyond the Guidelines: Treatment of Allergic Bronchopulmonary Aspergillosis in Cystic Fibrosis

Objective: To review the available literature addressing alternative allergic bronchopulmonary aspergillosis (ABPA) treatment options for patients with cystic fibrosis (CF). Data Sources: A literature search of PubMed was performed (January 2002 to April 2021) using the following search terms: allergic bronchopulmonary aspergillosis, aspergillus-related lung disease, cystic fibrosis. Manufacturer prescribing information, clinical practice guidelines, and data from ClinicalTrials.gov were incorporated in the reviewed data. Study Selection and Data Extraction: Relevant English-language studies or those conducted in humans were considered for inclusion. Data Synthesis: Available literature for alternative ABPA treatments in CF is lacking randomized controlled trials, but there is considerable support in case reports and case series describing the benefits in pediatric and adult patients. Recent literature has begun to explore the place in therapy for novel, corticosteroid-sparing treatment approaches. The alternative therapies summarized in this review all resulted in clinical improvement and subsequent discontinuation or dose reductions of oral corticosteroids, with minimal reported adverse drug effects. Relevance to Patient Care and Clinical Practice: Although corticosteroids are the cornerstone of ABPA management, the toxicities can be significant limitations in an already high-risk patient population. Patients may fail or become intolerant to guideline-recommended therapies and require alternative treatment approaches. Conclusions: Alternative treatment modalities for ABPA in patients with CF, including azole antifungals, pulsed intravenous glucocorticoids, omalizumab, mepolizumab, and inhaled amphotericin, appear to be efficacious and well tolerated. Pharmacological properties including route of administration, storage and stability, beyond use dating, and adverse effects of the various treatment modalities must be considered when selecting a practical care plan for patients.