CXCL8 (IL-8) and its receptors CXCR1 and CXCR2 are upregulated in pulmonary arterial hypertension, with CXCL8 promoting the recruitment and activation of inflammatory cells through binding to CXCR1/2, exacerbating pulmonary artery inflammation and remodeling. Here, CXCR1 is linked to pulmonary arterial hypertension.