Patients with pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc) who were treated with macitentan had similar therapeutic and safety results as patients with PAH from both idiopathic PAH (IPAH) and heritable PAH (HPAH), which is caused by a genetic mutation that runs in families, according to Vallerie McLaughlin, MD, FCCP, from the University of Michigan, Ann Arbor, who presented the study findings at the CHEST Annual Meeting 2019 in New Orleans.

Patients with PAH associated with connective tissue disease, including systemic sclerosis, are not expected to be as healthy as patients with other forms of PAH, according to the new research.

To evaluate macitentan’s impact on (PAH-SSc), the researchers used data from OPsumit USers (OPUS) Registry and OPsumit Historical USers cohort study (OrPHeUS), which provides medical information on patients with PAH who are newly started on macitentan. By October 2018, 659 patients with PAH-SSc had follow-up data in OPUS/OrPHeUS. The investigators compared these outcomes with those of 2,283 patients with IPAH and HPAH.

The researchers concluded: “In this real-world data set, the clinical outcomes and safety profile in patients with PAH-SSc are generally consistent with those of IPAH and HPAH patients. These data add to the body of evidence supporting the use of macitentan for the treatment of PAH-SSc.”

“Treatment with macitentan in this high-risk group of patients with pulmonary arterial hypertension with systemic sclerosis had similar outcomes compared with patients with idiopathic PAH,” Victor Test, MD, co-chair of the CHEST Scientific Program Committee and professor of Texas Tech University Health Sciences Center, says in a statement. “This result is encouraging for this high-risk group in a real-world setting.”