OrPHeUS was a retrospective, US, multicenter medical chart review (October 2013–March 2017). The characteristics, treatment patterns, safety, and outcomes during macitentan treatment of patients with CTD-PAH and its subgroups systemic sclerosis (SSc-PAH), systemic lupus erythematosus (SLE-PAH), and...
Patients with mild or moderate PAH exhibited a much better response to treatment than patients with severe PAH. SSc patients had worse clinical manifestations and haemodynamic parameters than SLE patients at the baseline and after treatment. The mortality rate in CTD-PAH patients was significantly ...