{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T07:51:15Z","timestamp":1790927475427,"version":"4.1.0"},"reference-count":24,"publisher":"European Respiratory Society (ERS)","issue":"4","content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2017,7,27]]},"published-print":{"date-parts":[[2017,10]]},"abstract":"<jats:p>Studies reporting the effects of modern strategies with pulmonary arterial hypertension (PAH)-targeted therapies in sarcoidosis-associated pulmonary hypertension (S-APH) are limited.<\/jats:p><jats:p>Clinical and haemodynamic data from newly diagnosed patients with severe S-APH (mean pulmonary artery pressure (mPAP) &gt;35\u2005mmHg or mPAP 25\u201335\u2005mmHg with cardiac index &lt;2.5\u2005L\u00b7min<jats:sup>\u22121<\/jats:sup>\u00b7m<jats:sup>\u22122<\/jats:sup>) were collected from the French Pulmonary Hypertension Registry between 2004 and 2015.<\/jats:p><jats:p>Data from 126 patients with severe S-APH were analysed (mean\u00b1<jats:sc>sd<\/jats:sc>age 57.5\u00b111.6\u2005years, 74% radiological stage IV). 97 patients (77%) received PAH-targeted therapy and immunosuppressive therapy was initiated or escalated in 33 patients at the time of pulmonary hypertension diagnosis. Four\u2005months after PAH-targeted therapy initiation, mean\u00b1<jats:sc>sd<\/jats:sc>pulmonary vascular resistance decreased from 9.7\u00b14.4 to 6.9\u00b13.0\u2005Wood units (p&lt;0.001), without significant improvement in exercise capacity. Among the 11 patients treated only with immunosuppressive therapy, a haemodynamic improvement was observed in four patients, including two with compressive lymph nodes. After a median follow-up of 28\u2005months, 39 patients needed PAH-targeted therapy escalation, nine underwent lung transplantation and 42 had died. Survival at 1, 3 and 5\u2005years was 93%, 74% and 55%, respectively.<\/jats:p><jats:p>PAH-targeted therapy improved short-term pulmonary haemodynamics in severe S-APH without change in exercise capacity. Immunosuppressive therapy improved haemodynamics in selected patients. 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