{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T18:03:45Z","timestamp":1792692225753,"version":"4.5.0"},"reference-count":27,"publisher":"European Respiratory Society (ERS)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2012,2,6]]},"published-print":{"date-parts":[[2012,10]]},"abstract":"<jats:p>Immune mechanisms and autoimmunity seem to play a significant role in idiopathic pulmonary arterial hypertension (IPAH) pathogenesis and\/or progression, but the pathophysiology is still unclear. Recent evidence has demonstrated a detrimental involvement of leptin in promoting various autoimmune diseases by controlling regulatory T-lymphocytes. Despite this knowledge, the role of leptin in IPAH is currently unknown. We hypothesised that leptin, synthesised by dysfunctional pulmonary endothelium, might play a role in the immunopathogenesis of IPAH by regulating circulating regulatory T-lymphocytes function.<\/jats:p><jats:p>First, we collected serum and regulatory T-lymphocytes from controls, and IPAH and scleroderma-associated pulmonary arterial hypertension (SSc-PAH) patients; secondly, we recovered tissue samples and cultured endothelial cells after either surgery or transplantation in controls and IPAH patients, respectively.<\/jats:p><jats:p>Our findings indicate that serum leptin was higher in IPAH and SSc-PAH patients than controls. Circulating regulatory T-lymphocyte numbers were comparable in all groups, and the percentage of those expressing leptin receptor was higher in IPAH and SSc-PAH compared with controls, whereas their function was reduced in IPAH and SSc-PAH patients compared with controls, in a leptin-dependent manner. Furthermore, endothelial cells from IPAH patients synthesised more leptin than controls.<\/jats:p><jats:p>Our data suggest that endothelial-derived leptin may play a role in the immunopathogenesis of IPAH.<\/jats:p>","DOI":"10.1183\/09031936.00159911","type":"journal-article","created":{"date-parts":[[2012,2,24]],"date-time":"2012-02-24T03:59:04Z","timestamp":1330055944000},"page":"895-904","source":"Crossref","is-referenced-by-count":126,"title":["Leptin and regulatory T-lymphocytes in idiopathic pulmonary arterial 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