• V Genin, D Horeau-Langlard, E Diot, F Gagnadoux, C Lavigne, M Fournet, C Durant, and C Agard.
• Service de médecine interne, Hôtel-Dieu, CHU de Nantes, centre de compétence HTAP de l'adulte, 1, place Alexis-Ricordeau, 44000 Nantes, France. Electronic address: victor.genin@chu-nantes.fr.
• Rev Med Interne. 2021 Dec 1; 42 (12): 825-831.

IntroductionPulmonary arterial hypertension (PAH) is a severe complication of connective tissue disease (CTD). Data on use of prostanoids in this particular subset of patients are lacking. We aimed to describe the characteristics of patients with PAH-CTD treated with prostanoids and the outcomes under treatment.MethodsIn this multicenter retrospective study, all patients treated with prostanoids since 2006 were included. Data on PAH and CTD were collected at the time of prostanoid introduction and under treatment.ResultsTwenty-one patients were included, of whom 20 (95%) had limited cutaneous systemic sclerosis. Nineteen patients were treated with oral monotherapy or combination before addition of prostanoid. Treprostinil was the most used molecule (57% of patients). At the time of prostanoid introduction, 90% of patients were considered at high risk for death. Among patients who had right heart catheterization during follow-up, there was no significant difference in haemodynamics. No extrarespiratory worsening of the CTD was reported. The 1-year survival under prostanoid was 62%. In univariate analysis, NYHA functional class was associated with survival under treatment.ConclusionThis study provides original data on use of prostanoids in a cohort consisting mainly of systemic sclerosis. It underlines the difficulty to achieve a standardized assessment in this subset of patients. Safety profile was comparable with data reported in idiopathic PAH.Copyright © 2021. Published by Elsevier Masson SAS.

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