P174 Human epididymis protein 4 (HE4) plasma concentrations correlate with the improvement of ppFEV1 in response to LUM/IVA therapy in people with cystic fibrosis homozygous for p.Phe508del-CFTR

We have previously documented that elevated plasma HE4 concentrations decreased in people with CF (pwCF) bearing p.Gly551Asp-CFTR mutation in response to ivacaftor (IVA), and these concentrations were inversely correlated with ppFEV1. Although the PROSPECT study has evaluated the effectiveness of lumacaftor (LUM)/IVA in pwCF homozygous for p.Phe508del-CFTR variant, plasma biomarkers have not been used to monitor treatment efficacy.