biostudies-literatureLaverty SLCystic Fibrosis Trust106131https://www.ebi.ac.uk/biostudies/studies/S-EPMC12878693biostudies-literatureUnknown124<h4>Background</h4>The impact of CFTR modulator therapy on host immunity and outcomes in people with Cystic Fibrosis (CF)-related Aspergillus lung disease is poorly defined. We aimed to characterise fungal-relevant clinical outcomes post-CFTR modulators and assess effects on the Aspergillus-dependent Type I/III interferome.<h4>Methods</h4>Biomarkers of Aspergillus-related lung disease (Aspergillus-specific IgE/IgG), anti-fungal and corticosteroid therapy were analysed in a retrospective cohort of people with CF pre and post Elexacaftor/Tezacaftor/Ivacaftor (ETI) modulator therapy. Homozygous F508del (CF) and CFTR TALEN-corrected bronchial epithelial cells (BECs) were challenged with Aspergillus conidia and hyphae in the presence or absence of ETI CFTR modulator therapy with bulk RNA transcEBioMedicineCFTR modulators partially restore the epithelial interferome in Aspergillus infection to improve clinical outcome.PMC12878693SRC015Hopwood BShah AFelton ILaverty SLArmstrong-James DKelleher PCasey MSimmonds NfalseCFTR modulators partially restore the epithelial interferome in Aspergillus infection to improve clinical outcome.<h4>Background</h4>The impact of CFTR modulator therapy on host immunity and outcomes in people with Cystic Fibrosis (CF)-related Aspergillus lung disease is poorly defined. We aimed to characterise fungal-relevant clinical outcomes post-CFTR modulators and assess effects on the Aspergillus-dependent Type I/III interferome.<h4>Methods</h4>Biomarkers of Aspergillus-related lung disease (Aspergillus-specific IgE/IgG), anti-fungal and corticosteroid therapy were analysed in a retrospective cohort of people with CF pre and post Elexacaftor/Tezacaftor/Ivacaftor (ETI) modulator therapy. Homozygous F508del (CF) and CFTR TALEN-corrected bronchial epithelial cells (BECs) were challenged with Aspergillus conidia and hyphae in the presence or absence of ETI CFTR modulator therapy with bulk RNA transc2026-01-01T00:00:00Z2026 Feb2026-07-15T13:15:48.449Z2026-07-05T03:08:11.693ZS-EPMC128786934162140610.1016/j.ebiom.2026.106131