In the EDITA trial, patients with systemic sclerosis (SSc) and mild pulmonary vascular disease (PVD) treated with ambrisentan had a significant decline of pulmonary vascular resistance (PVR) but not of mean pulmonary arterial pressure (mPAP) vs. placebo after six months. The EDITA-ON study aimed to assess long-term effects of open label therapy with ambrisentan vs. no pulmonary arterial hypertension (PAH) therapy. Patients who participated in the EDITA study and received regular follow-up were included in EDITA-ON. Clinical, echocardiographic, laboratory, exercise and hemodynamic parameters during follow-up were analysed. The primary endpoint was to assess whether continued treatment with ambrisentan vs. no treatment prevented the development of PAH according to the new definition. Of 38 SSc patients included in the EDITA study four were lost to follow-up. Of the 34 remaining patients (age 55 ± 11 years, 82.1% female subjects), 19 received ambrisentan after termination of the blinded phase, 15 received no PAH medication. The mean follow-up time was 2.59 ± 1.47 years, during which 29 patients underwent right heart catheterization. There was a significant improvement of mPAP in catheterised patients receiving ambrisentan vs. no PAH treatment (-1.53 ± 2.53 vs. 1.91 ± 2.98 mmHg, p = 0.003). In patients without PAH treatment 6/12 patients had PAH vs. 1/17 of patients receiving ambrisentan (p < 0.0001). In SSc patients with early PVD, the development of PAH and/or deterioration was less frequent among patients receiving ambrisentan, indicating that early treatment and close follow-up could be beneficial in this high-risk group. Future trials in this field are needed to confirm these results.

中文翻译：

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安立生坦对系统性硬化症和轻度肺动脉高压患者的影响：来自 EDITA 研究的长期随访数据


在 EDITA 试验中，与安慰剂相比，系统性硬化症 (SSc) 和轻度肺血管疾病 (PVD) 患者接受安立生坦治疗后，肺血管阻力 (PVR) 显着下降，但平均肺动脉压 (mPAP) 没有显着下降。 EDITA-ON 研究旨在评估使用 ambrisentan 的开放标签疗法与不使用肺动脉高压 (PAH) 疗法的长期效果。 EDITA-ON 纳入了参加 EDITA 研究并接受定期随访的患者。分析随访期间的临床、超声心动图、实验室、运动和血流动力学参数。主要终点是根据新定义评估继续使用安立生坦治疗与不治疗是否可以预防 PAH 的发生。 EDITA 研究中纳入的 38 名 SSc 患者中，有 4 名患者失访。其余 34 名患者（年龄 55 ± 11 岁，82.1% 女性受试者）中，19 名患者在盲法阶段结束后接受了安立生坦治疗，15 名患者未接受 PAH 药物治疗。平均随访时间为2.59±1.47年，期间29名患者接受了右心导管检查。接受安立生坦治疗的插管患者与未接受 PAH 治疗的患者相比，mPAP 显着改善（-1.53​​ ± 2.53 对比 1.91 ± 2.98 mmHg，p = 0.003）。在未接受 PAH 治疗的患者中，6/12 名患者患有 PAH，而接受安立生坦治疗的患者为 1/17 (p < 0.0001)。在患有早期PVD的SSc患者中，接受ambrisentan的患者发生PAH和/或恶化的频率较低，这表明早期治疗和密切随访可能对这一高危人群有益。需要该领域的未来试验来证实这些结果。