Early treatment with ambrisentan might prevent PAH development in patients with SSc

Continuing treatment with ambrisentan showed potential in preventing pulmonary arterial hypertension (PAH) in participants with systemic sclerosis (SSc) and borderline PAH. These findings from the EDITA study highlight ambrisentan's potential in managing early-stage pulmonary vascular disease in SSc.

PAH is a rare disease that entails a rise in vascular resistance that puts a strain on the right ventricle [1]. “Patients with SSc are at very high risk to develop PAH at any time of their disease, with a prevalence of 10 to 12%, and they may progress rapidly,” Dr Panagiota Xanthouli (Heidelberg University Hospital, Germany) pointed out [2].

The previously published phase 2 EDITA study tested the efficacy of ambrisentan on mean pulmonary arterial pressure (mPAP) in 38 participants with SSc and mildly elevated PAH [3]. Its primary endpoint of difference in mPAP change was not met, but a significant decrease in the secondary endpoint of 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. Dr Xanthouli presented the long-term follow-up results [2].

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 catheterisation.

The results revealed a significant difference in the amelioration of mPAP in favour of the ambrisentan treatment (-1.53 vs +1.91 mmHg without treatment; P=0.003). This was mirrored by no newly diagnosed PAH in the ambrisentan group but 4 new cases of PAH with mPAP above 20 mmHg in the control group (P<0.0001). The changes in PVR showed a heterogeneous picture without a statistically significant difference between the groups. There were 3 cases of deterioration and no cases of 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 preventing progress of pulmonary vascular disease and development of PAH according to the new definition,” Dr Xanthouli summarised.

1. Luna-López R, et al. Med Clin (Barc). 2022;158:622-629.
2. 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, EULAR 2024 Congress, 12–15 June, Vienna, Austria.
3. Pan Z, et al. Arthritis Res Ther. 2019;21(1):217.

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Posted on 29 July 2024