PT  - JOURNAL ARTICLE
AU  - Athénaïs Boucly
AU  - Vincent Cottin
AU  - Hilario Nunes
AU  - Xavier Jaïs
AU  - Abdelatif Tazi
AU  - Grégoire Prévôt
AU  - Martine Reynaud-Gaubert
AU  - Claire Dromer
AU  - Catherine Viacroze
AU  - Delphine Horeau-Langlard
AU  - Christophe Pison
AU  - Emmanuel Bergot
AU  - Julie Traclet
AU  - Jason Weatherald
AU  - Gérald Simonneau
AU  - Dominique Valeyre
AU  - David Montani
AU  - Marc Humbert
AU  - Olivier Sitbon
AU  - Laurent Savale
TI  - Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension
AID  - 10.1183/13993003.00465-2017
DP  - 2017 Oct 01
TA  - European Respiratory Journal
PG  - 1700465
VI  - 50
IP  - 4
4099  - http://erj.ersjournals.com/content/50/4/1700465.short
4100  - http://erj.ersjournals.com/content/50/4/1700465.full
SO  - Eur Respir J2017 Oct 01; 50
AB  - 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.Severe pulmonary hypertension remains a life-threatening complication of sarcoidosis in the modern management era http://ow.ly/fIln30etYkE