Key clinical point: Macitentan produced similar outcomes in patients with pulmonary arterial hypertension associated with systemic sclerosis (PAH-SSc) and those with idiopathic PAH (IPAH) or heritable PAH (HPAH).
Major finding: About 39% of patients in both groups discontinued macitentan, hepatic adverse events occurred in 7.4% of IPAH/HPAH patients and 7.9% of PAH-SSc patients, and the estimated 12-month survival was 92.9% in the IPAH/HPAH group and 91.3% in the PAH-SSc group.
Study details: Data from the prospective OPUS registry and retrospective OrPHeUS study on 2,311 patients with IPAH/HPAH and 668 patients with PAH-SSc.
Disclosures: The OPUS registry and OrPHeUS study are sponsored by Actelion. Dr. McLaughlin disclosed relationships with Actelion, Acceleron, Bayer, Caremark, CiVi Biopharma, Reata, Sonovie, and United Therapeutics.
McLaughlin V et al. CHEST 2019. Abstract, doi: 10.1016/j.chest.2019.08.827.