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Macitentan safe, effective for PAH associated with systemic sclerosis
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NEW ORLEANS — New real-world data demonstrate safety and effectiveness of macitentan for the treatment of pulmonary arterial hypertension associated with systemic sclerosis, with outcomes consistent with those in idiopathic and heritable PAH, researchers reported at the CHEST Annual Meeting.
Using the OPUS Registry and OrPHeUS cohort study, which provide information on patients with PAH who are newly started on macitentan (Opsumit, Actelion Pharmaceuticals), researchers analyzed follow-up data on 659 patients with PAH associated with systemic sclerosis. The researchers then compared outcomes in this population with those of 2,283 patients with idiopathic PAH or heritable PAH.
Treatment discontinuation was reported in 30.2% of patients with PAH associated with systemic sclerosis and 32.8% of patients with idiopathic and heritable PAH. In each group, discontinuation was related to an adverse event in about 17% of patients. Alanine aminotransferase (ALT) or aspartate aminotransferase (AST) three or more times the upper limit of normal occurred in 3.9% and 2.7%, respectively, and ALT/AST three or more times the upper limit of normal and total bilirubin two or more times the upper limit of normal occurred in 0.5% and 0.3%, respectively, according to the results.
At 12 months, the Kaplan-Meier survival estimate was 92% for patients with PAH associated with systemic sclerosis and 93% for those with idiopathic and heritable PAH.
One or more hospitalizations occurred in 40% of patients with PAH associated with systemic sclerosis and 36.3% with idiopathic and heritable PAH, according to the results.
At initiation of macitentan, patients with PAH associated with systemic sclerosis had a median age of 64 years, nearly three-quarters had WHO functional class III or IV PAH, 61% were receiving another PAH therapy and 45% had been diagnosed within 6 months. Overall median exposure to macitentan was 14.7 months among patients with PAH associated with systemic sclerosis and 13.2 months in those with idiopathic or heritable PAH.
“These data add to the body of evidence supporting the use of macitentan for the treatment of PAH-systemic sclerosis,” Vallerie McLaughlin, MD, FCCP, director of the pulmonary hypertension program and the Kim A. Eagle MD Endowed Professor of Cardiovascular Medicine at University of Michigan, and colleagues concluded. – by Katie Kalvaitis
Reference:
McLaughlin V. Pulmonary Hypertension: Characteristics and Treatment. Presented at: CHEST Annual Meeting; Oct. 19-23, 2019; New Orleans.
Disclosure: McLaughlin reports she consults for Acceleron, Actelion, Arena, Bayer, Caremark and United Therapeutics, and receives research grants from Acceleron, Actelion, Arena, Bayer, Gilead and Sonovie.