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Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension

Abstract : Studies reporting the effects of modern strategies with pulmonary arterial hypertension (PAH)-targeted therapies in sarcoidosis-associated pulmonary hypertension (S-APH) are limited.Clinical and haemodynamic data from newly diagnosed patients with severe S-APH (mean pulmonary artery pressure (mPAP) >35 mmHg or mPAP 25-35 mmHg with cardiac index <2.5 L·min-1·m-2) were collected from the French Pulmonary Hypertension Registry between 2004 and 2015.Data from 126 patients with severe S-APH were analysed (mean±sd age 57.5±11.6 years, 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 months after PAH-targeted therapy initiation, mean±sd pulmonary vascular resistance decreased from 9.7±4.4 to 6.9±3.0 Wood units (p<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 months, 39 patients needed PAH-targeted therapy escalation, nine underwent lung transplantation and 42 had died. Survival at 1, 3 and 5 years was 93%, 74% and 55%, respectively.PAH-targeted therapy improved short-term pulmonary haemodynamics in severe S-APH without change in exercise capacity. Immunosuppressive therapy improved haemodynamics in selected patients. Pulmonary hypertension in sarcoidosis remains associated with a poor prognosis.
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Contributeur : Sarah Hamant <>
Soumis le : vendredi 21 décembre 2018 - 10:53:25
Dernière modification le : mercredi 16 septembre 2020 - 17:13:30

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Athénaïs Boucly, Vincent Cottin, Hilario Nunes, Xavier Jaïs, Abdelatif Tazi, et al.. Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension. European Respiratory Journal, European Respiratory Society, 2017, 50 (4), pp.1700465. ⟨10.1183/13993003.00465-2017⟩. ⟨hal-01963157⟩



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