Early Treatment With Ambrisentan Prevents PAH Development in Patients With SSc

Jul 16, 2024

Photo Credit: Design Cells

In SSc plus mild pulmonary vascular disease, early treatment with ambrisentan effectively prevented pulmonary vascular disease progress and development of PAH.

Long-term follow-up data from the EDITA study showed that participants with systemic sclerosis (SSc) and borderline pulmonary arterial hypertension (PAH) who continued ambrisentan developed significantly less PAH than participants on standard-of-care alone.

The primary phase 2 EDITA study tested the efficacy of ambrisentan on mean pulmonary arterial pressure (mPAP) in 38 participants with SSc and mildly elevated PAH. Its primary endpoint of difference in mPAP change was not met, but among the secondary endpoints, a significant decrease in pulmonary vascular resistance (PVR) was observed within the ambrisentan group. To investigate this phenomenon further, 34 EDITA participants who had continued regular clinical visits were evaluated for the possible effect of continued ambrisentan on the prevention of PAH according to its 2022 ESC/ERS definition. Panagiota Xanthouli, MD, presented the long-term follow-up results.

At the end of EDITA, 15 participants continued without PAH treatment, and 19 stayed on ambrisentan. During a mean follow-up time of 2.59 years after EDITA, 29 participants underwent right heart catheterization.

The results revealed a significant difference in the amelioration of mPAP in favor of the ambrisentan treatment (-1.53 vs 1.91 mmHg; P=0.003). No newly diagnosed PAH mirrored this in the ambrisentan group, but 4 cases of PAH with mPAP above 20 mmHg in the control group (P<0.001). The changes in PVR showed a heterogeneous picture without a statistically significant difference between the groups. There were 3 cases of deterioration and no improvement in the control group compared with 2 cases of deterioration and 3 cases of improvement on ambrisentan.

“In patients with SSc and a mild form of pulmonary vascular disease, early treatment with ambrisentan was highly effective in terms of prevention of progress of pulmonary vascular disease and development of PAH according to the new definition,” Dr. Xanthouli summarized.

Author

Teresa Sellinger

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REFERENCES & ADDITIONAL READING

Pan Z, et al. Early treatment with ambrisentan of mildly elevated mean pulmonary arterial pressure associated with systemic sclerosis: a randomized, controlled, double-blind, parallel group study (EDITA study). Arthritis Res Ther. 2019;21(1):217. Published 2019 Oct 26. doi:10.1186/s13075-019-1981-0

Xanthouli P, et al. Effect of treatment with ambrisentan in patients with systemic sclerosis and mild pulmonary arterial hypertension: long-term follow-up data from EDITA study. OP0241. Presented at: EULAR 2024, June 12-15, Vienna, Austria.