Immune Checkpoint Regulation of Pulmonary Fibrosis [bulk RNA-seq]

Fibroblasts from idiopathic pulmonary fibrosis (IPF) patients acquire an invasive phenotype that is essential for progressive fibrosis. The immune checkpoint ligand CD274 (PD-L1) is up-regulated on invasive lung fibroblasts, regulated by P53 and FAK signaling, and drives lung fibrosis in a humanized IPF model in mice. Targeting CD274high fibroblasts blunted invasion in vitro and attenuated fibrosis in vivo, suggesting that CD274 may be a novel therapeutic target in IPF. Reveal of the expression of the checkpoint PD1 ligand CD274 in invasive and non-invasive fibroblasts of both normal human lungs and IPF lungs