Intravenous Prostanoids in Systemic Sclerosis-Associated Pulmonary Arterial Hypertension

A Single-Centre Experience

Maria Vlachou; Hossam Fayed; Adele Dawson; Sally Reddecliffe; Alexander Stevenson; Ross J Thomson; Benjamin Emmanuel Schreiber; J. Gerry Coghlan


Rheumatology. 2022;61(3):1106-1114. 

In This Article


Survival among SSc-PAH patients deteriorating despite oral therapy remains poor in the UK despite i.v. prostanoid therapy. In SSc-PAH patients on i.v. prostanoids, ESC score and REVEAL score at baseline and at first formal reassessment identifies patients with a particularly poor prognosis. Potential explanations include delayed initiation of treatment and inadequate use of triple therapy among this high-risk group.