In the lungs of PAH patients and chronic hypoxic mice, Looney et al. reported that the expression of FLI-1 and ERG were downregulated, resulting in the significant upregulation of the expression of proinflammatory genes (CXCL10, HAS2, IL1A, CCL5, CXCL11 and VCAM1) and IFN-response genes (GBP4, IFIT2, OASL and IRF6) and the downregulation of the expression of regulatory endothelial homeostasis genes (FABP4, LYVE-1, and APLN), leading to spontaneous lung inflammation and pulmonary vascular diseases [67]. Here, FLI1 is linked to pulmonary arterial hypertension.