Chronic lung inflammation in cystic fibrosis (CF) often persists even after treatment with newly-approved gene therapies or small molecule CFTR modulators—an unresolved clinical paradox. A new study published in EXO – Beyond the Cell identifies a potential explanation: inflammation is driven not only by intrinsic defects in immune cells, but also by lasting changes in the lung microenvironment.
Using induced pluripotent stem cell (iPSC)-derived macrophages from both healthy donors and CF patients, researchers from Gordana Vunjak-Novakovic’s team at Columbia University developed an all-human, in vitro model to disentangle these effects. By combining patient-derived immune cells with decellularized extracellular matrix (ECM) from end-stage CF lungs, the study separates cell-intrinsic and microenvironmental contributions to disease… Continue reading.