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Abstract：
Selexipag is indicated for the treatment of pulmonary arterial hypertension (PAH), including PAH associated with connective tissue disease (CTD), and further insights into the management of selexipag-treated PAH-CTD patients in clinical settings are needed. These analyses of the ongoing, multicenter, prospective EXPOSURE (EUPAS19085) study describe characteristics, treatment patterns, tolerability, and outcomes of PAH-CTD patients initiating selexipag in Europe/Canada. All analyses were descriptive, with idiopathic PAH patients who typically display better prognosis included for context. Six hundred ninety-eight selexipag-treated patients had follow-up information; 178 (26%) had PAH-CTD. The median age was 68 years, patients were predominantly female (88%), and with WHO functional class III symptoms (63%); the median time since diagnosis was 1.7 years. There were 5% patients at low, 25% intermediate-low, 40% intermediate-high, and 30% high risk of 1-year mortality, according to the ESC/ERS 4-strata risk score. Most (80%) initiated selexipag as a triple oral therapy, and most of these (62%) remained on triple therapy 6 months post-baseline. Over a median (Q1-Q3) selexipag exposure period of 8.6 (2.5-17.2) months, 79 (44%) patients discontinued selexipag; 36 (20%) due to tolerability/adverse events. Sixty (34%) patients were hospitalized at least once; 120 hospitalizations occurred, with 49 (48%) deemed PAH-related. Survival at 1 year was 85%, and at 2 years was 71%; 29 (16%) patients died. These results describe the use of combination therapy with selexipag for patients with PAH-CTD. These findings suggest an opportunity to optimize the benefits of selexipag among patients with PAH-CTD by moving from escalating after years in response to clinical deterioration to escalating sooner to prevent clinical deterioration.
摘要：
Selexipag用于治疗肺动脉高压（PAH），包括与结缔组织病（CTD）相关的PAH，在临床环境中需要进一步了解Selexipag治疗的PAH-CTD患者的管理。这些分析正在进行的，多中心，前瞻性暴露(EUPAS19085)研究描述了特征，治疗模式,耐受性,以及在欧洲/加拿大开始自检的PAH-CTD患者的结局。所有分析都是描述性的，特发性PAH患者通常表现出更好的预后。六百九十八名接受自我治疗的患者获得了随访信息；178名（26％）患有PAH-CTD。中位年龄为68岁,患者主要为女性（88%），并伴有WHO功能III类症状(63%);自诊断以来的中位时间为1.7年.有5%的患者处于低位，25%中低,40%中高,和30%的1年死亡率高风险，根据ESC/ERS4层风险评分。大多数（80％）开始将selexipag作为三联口服疗法，其中大多数(62%)在基线后6个月仍接受三联疗法.在中位数（Q1-Q3）8.6（2.5-17.2）个月的Selexipag暴露期，79例（44％）患者因耐受性/不良事件而停止了Selexipag；36例（20％）。60例（34%）患者至少住院一次；120例住院，49（48%）被认为与PAH相关。1年生存率为85%，2年时为71%;29例(16%)患者死亡。这些结果描述了对PAH-CTD患者使用selexipag联合治疗。这些发现表明，通过从多年后对临床恶化的反应升级到更快地升级以防止临床恶化，可以优化PAH-CTD患者selexipag的益处。
