A recent study evaluated the prevalence of anti-ETBR antibodies in SSc patients, finding more elevated serum levels in SSc-PAH patients vs. those of healthy controls [31]; given the immunomodulatory role of ETBR, it could be hypothesised that these autoantibodies can block its downstream signalling pathway, thus enhancing inflammation, but more studies are required to ascertain their exact pathogenetic role. This evidence concerns the gene EDNRB and pulmonary arterial hypertension.