Advances in the Diagnosis and Management of Allergic Bronchopulmonary Aspergillosis/Mycosis.

Abstract

Allergic bronchopulmonary aspergillosis/mycosis (ABPA/ABPM) is an immunologically mediated pulmonary disorder triggered by the colonization of filamentous fungi in the airways of patients with asthma or cystic fibrosis, leading to type I and III hypersensitivity responses, eosinophilic mucus plug formation, and central bronchiectasis. Over the past decade, advances in basic and clinical research have substantially reshaped the understanding and management of this disease. Although Aspergillus species, particularly Aspergillus fumigatus, remain the predominant causative fungi, epidemiological studies have identified Schizophyllum commune as an important and previously underrecognized pathogen responsible for ABPM. A major breakthrough in elucidating disease pathophysiology is the recognition of eosinophil extracellular trap cell death as a central mechanism driving highly viscous mucus impaction. Diagnostic criteria with improved accuracy and standardized treatment protocols have been established through randomized controlled trials, culminating in the 2024 revision of the International Society for Human and Animal Mycology guidelines. However, significant unmet needs remain. Despite high initial response rates to corticosteroids or azole antifungals, nearly half of patients fail to achieve remission. Predictors of treatment failure include heightened A. fumigatus-specific sensitization and radiological evidence of eosinophilic mucus impaction. Biologic agents targeting type 2 inflammation have emerged as promising options for difficult-to-treat disease, although their efficacy in acute ABPA is still uncertain. Frequent exacerbations also pose a major challenge, and the potential benefits of maintenance inhaled antifungals and environmental interventions are under investigation. Collectively, advances in pathophysiology, diagnostics, and therapeutics are transforming ABPA/ABPM management, yet substantial gaps persist in improving long-term outcomes. Trial Registration: ClinicalTrials.gov Identifier: NCT04442269.