背景:风险评估有助于肺动脉高压(PAH)的管理和临床决策。此分析描述了特征,PAH患者的治疗模式和结果,根据临床环境中使用selexipag治疗升级时的风险状态进行分类。
方法:患者在正在进行的多中心开始自列分析,前瞻性暴露(EUPAS19085)研究被分组为低,中低,根据ESC/ERS4层方法,1年死亡率的中高风险或高风险。
结果:截至2022年11月,77%(535/698)的患者开始进行自exipag的数据允许进行风险计算;14%(N=76)低,31%(N=168)中低,34%(N=182)中高和20%(N=109)1年死亡率的高风险。总的来说,患者以女性为主(71%),特发性/遗传性PAH(56%)或与结缔组织疾病相关的PAH(CTD-PAH;27%),中位年龄60岁和流行(2岁)疾病。从低到高风险,CTD-PAH的比例和年龄增加(从12%-40%和46-68岁,分别);从诊断开始的时间减少,心血管危险因素的存在增加。风险组的大多数患者(74-81%)开始使用selexipag作为三联口服联合治疗的一部分。总体中位数(Q1,Q3)Selexipag暴露持续时间为10.1(3.5,24.1)个月。住院患者的比例随着风险组的增加而增加(16-42%从低到高,分别);与其他风险组(47-54%)相比,高风险组(71%)与PAH相关的住院治疗更多。Kaplan-Meier生存率估计为98%,98%,93%和80%在1年期和98%,92%,81%和67%在2年,从低到高风险,分别。
结论:在临床环境中,selexipag在所有风险群体中启动,主要作为三联疗法。只有45%的患者在selexipag开始时处于低/中-低风险,这表明有机会进行更频繁的患者监测和更早的治疗升级。考虑到4层风险评估是当代PAH队列中住院和生存的预后因素.本文提供了图形摘要。
肺动脉高压(PAH)是一种随时间恶化的疾病。要决定治疗,我们需要知道疾病的阶段。我们可以通过测量患者在未来几年的死亡风险来做到这一点。Selexipag是一种治疗PAH的药物。该分析包括居住在欧洲和加拿大的患者,他们开始使用selexipag治疗他们的PAH疾病。我们的发现表明,可以改善对患者健康状况的监测和开始selexipag的时机。这项分析包括698名患者参加了暴露研究(EUPAS19085),它着眼于PAH患者的现实生活中的治疗。总的来说,71%的患者是女性,中位年龄为60岁,大多数人已被诊断为PAH约2年,并且已经服用了另外两种治疗PAH的药物。在Selexipag治疗开始时,14%的患者被归类为低风险,31%为中低风险,34%为中高风险,20%为明年的高风险。与低风险组相比,更多的高风险患者住院。治疗1年后,低(98%)和中-低组(98%)存活的患者多于中-高(93%)和高风险组(80%).用selexipag治疗2年后也是如此(98%,92%,81%和67%,分别)。这项研究证实,评估患者的风险水平可以表明他们随着时间的推移会做得如何,并表明早期使用selexipag进行治疗应被认为有可能预防疾病的恶化。
BACKGROUND: Risk assessment can aid management of pulmonary arterial hypertension (PAH) and clinical decision-making. This analysis describes characteristics, treatment patterns and outcomes of patients with PAH, categorised by risk status at time of treatment escalation with selexipag in clinical settings.
METHODS: Patients initiating selexipag in the ongoing multicentre, prospective EXPOSURE (EUPAS19085) study were grouped as low, intermediate-low, intermediate-high or high risk of 1-year mortality according to the ESC/ERS 4-strata method.
RESULTS: As of November 2022, 77% (535/698) of patients initiating selexipag had data allowing for risk calculation; 14% (N = 76) were low, 31% (N = 168) intermediate-low, 34% (N = 182) intermediate-high and 20% (N = 109) high risk of 1-year mortality. Overall, patients were predominantly female (71%), with idiopathic/heritable PAH (56%) or PAH associated with connective tissue disease (CTD-PAH; 27%), median age of 60 years and prevalent (2 years) disease. From low to high risk, proportion of CTD-PAH and age increased (from 12%-40% and 46-68 years, respectively); time from diagnosis decreased and presence of cardiovascular risk factors increased. Most patients across risk groups (74-81%) initiated selexipag as part of triple oral combination therapy. Overall median (Q1, Q3) selexipag exposure duration was 10.1 (3.5, 24.1) months. Proportions of hospitalised patients increased with increasing risk group (16-42% from low to high, respectively); more hospitalisations were PAH-related for the high risk (71%) versus other risk groups (47-54%). Kaplan-Meier survival estimates were 98%, 98%, 93% and 80% at 1-year and 98%, 92%, 81% and 67% at 2-years, from low to high risk, respectively.
CONCLUSIONS: In clinical settings, selexipag is initiated across all risk groups, predominantly as triple therapy. Only 45% of patients being at low/intermediate-low risk at selexipag initiation suggests an opportunity for more frequent patient monitoring and earlier treatment escalation, given that 4-strata risk assessment was prognostic for hospitalisations and survival in this contemporary PAH cohort. A graphical abstract is available with this article.
Pulmonary arterial hypertension (PAH) is a disease that gets worse over time. To make decisions about treatment, we need to know the stage of the disease. We can do this by measuring the patient’s risk of death during the next few years. Selexipag is a medication for PAH. This analysis included patients living in Europe and Canada who started treatment with selexipag for their PAH disease. Our findings suggest that the monitoring of patients’ health and the timing of starting selexipag can be improved. This analysis includes 698 patients taking part in the EXPOSURE study (EUPAS19085), which looks at the real-life treatment of patients with PAH. Overall, 71% of patients were female, the median age was 60 years, most had been diagnosed with PAH for around 2 years and were already taking two other medications for their PAH disease. At the beginning of selexipag treatment, 14% of patients were classified as low risk, 31% as intermediate-low risk, 34% as intermediate-high risk and 20% as high risk of mortality within the next year. More high-risk patients were hospitalised compared with the lower risk groups. After 1 year of treatment, more patients in the low (98%) and intermediate-low groups (98%) were alive than those in the intermediate-high (93%) and high risk groups (80%). The same was true after 2 years of treatment with selexipag (98%, 92%, 81% and 67%, respectively). This study confirms that assessing patients’ risk levels can indicate how well they will do over time and shows that earlier treatment with selexipag should be considered to potentially prevent worsening of the disease.