Effect of Elexacaftor/tezacaftor/ivacaftor on allergic bronchopulmonary aspergillosis in a paediatric cystic fibrosis population

Allergic Bronchopulmonary Aspergillosis (ABPA) is an airway allergic response to Aspergillus Fumigatus (AF) spores, affecting patients with cystic fibrosis (CF), with potential to cause extensive lung fibrosis. Elexacaftor/tezacaftor/ivacaftor (Kaftrio) acts to increase the number of CFTR channels and increase their activity to limit progression of CF. This retrospective study aimed to evaluate the effect of Kaftrio on ABPA in children with CF. Spirometry, total IgE, AF specific IgE, AF specific IgG (AFIgG), eosinophil count (EC), sputum culture results, and steroid, CFTR modulator, and antifungal use were collected from ≥ 4 annual reviews for 63 children treated with Kaftrio for ≥ 1 year. Effect of Kaftrio on continuous variables was evaluated using Welch two sample t-test. Subgroup analysis was carried out for children previously treated for ABPA (n = 8) and children not been treated with any CFTR modulator prior to Kaftrio (n = 21). While no significant differences were found amongst the total population, there was a significant decrease in AFIgG (p = 0.004, Figure 1) and EC (p = 0.04) in children previously treated for ABPA. Furtermore, no children on Kaftrio (n = 63) required treatment for ABPA or had sputum cultures positive for AF. <fig><object-id>erj;64/suppl_68/PA557/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig> Figure 1 - Progression of AFIgG in children treated for ABPA This study suggests Kaftrio may have additional benefits for children with ABPA.