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.
No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this article.
Data availability statement
Anonymized data underlying this article can be provided on reasonable request to the corresponding author.
Rheumatology. 2022;61(3):1106-1114. © 2022 Oxford University Press