Barnikel Michaela, Kneidinger Nikolaus, Klenner Friederike, Waelde Andrea, Arnold Paola, Sonneck Torben, Behr Jürgen, Neurohr Claus, Milger Katrin
1 Department of Internal Medicine V, Ludwig-Maximilians-University (LMU) of Munich, Munich, Germany.
2 Comprehensive Pneumology Center (CPC-M), Member of the German Center for Lung Research (DZL), Munich, Germany.
Pulm Circ. 2019 Jan-Mar;9(1):2045894019832199. doi: 10.1177/2045894019832199.
Selexipag is an orally available selective IP prostacyclin-receptor agonist licensed since 2016 for the therapy of pulmonary arterial hypertension (PAH). We aimed to describe real-life data of patients with pulmonary hypertension (PH) treated with selexipag. We analyzed all patients initiated with selexipag from July 2016 to April 2018 at the Department of Internal Medicine V, University of Munich. Non-invasive and invasive parameters corresponding to the risk assessment were collected at baseline and follow-up (FU). Furthermore, we recorded tolerability. Twenty-six patients were treated with selexipag, of whom 23 had PAH and three had chronic thromboembolic PH. At baseline, most patients were in function class (FC) II or III (42% and 54%, respectively). All patients were under medical treatment for PH, mostly dual therapy (92%). One or more side effects were noted in 19 patients, while seven reported no side-effects. FU assessment was available in 20 patients after 149 ± 80 days of treatment. Nt-proBNP (median, baseline 1641 pg/mL, FU 1185 pg/mL, P = 0.05) and PVR (mean ± SD, baseline 8.5 ± 4.3 WU, FU 5.6 ± 1.1 WU; P < 0.05) improved significantly. At FU, at least one risk assessment parameter improved in nine patients (45%), all parameters remained in the same risk group in seven patients (35%), and at least one parameter deteriorated in four patients (20%). Interestingly, patients with any side effect throughout the dose titration had a better treatment response than those without any side effects. In our real-life cohort, the majority of patients with PH treated with selexipag showed a stable or improved risk assessment at FU.
司来帕格是一种口服选择性前列环素受体激动剂，自2016年起被批准用于治疗肺动脉高压（PAH）。我们旨在描述接受司来帕格治疗的肺动脉高压（PH）患者的真实生活数据。我们分析了2016年7月至2018年4月在慕尼黑大学内科V接受司来帕格初始治疗的所有患者。在基线和随访（FU）时收集与风险评估相应的非侵入性和侵入性参数。此外，我们记录了耐受性。26例患者接受司来帕格治疗，其中23例患有PAH，3例患有慢性血栓栓塞性PH。在基线时，大多数患者处于功能分级（FC）II或III级（分别为42%和54%）。所有患者均接受PH药物治疗，大多数为联合治疗（92%）。19例患者出现一种或多种副作用，而7例报告无副作用。20例患者在治疗149±80天后进行了FU评估。N末端脑钠肽前体（中位数，基线1641 pg/mL，FU 1185 pg/mL，P = 0.05）和肺血管阻力（平均值±标准差，基线8.5±4.3 WU，FU 5.6±1.1 WU；P < 0.05）显著改善。在FU时，9例患者（45%）至少有一项风险评估参数改善，7例患者（35%）所有参数仍处于同一风险组，4例患者（20%）至少有一项参数恶化。有趣的是，在剂量滴定过程中出现任何副作用的患者比没有任何副作用的患者有更好的治疗反应。在我们的真实生活队列中，大多数接受司来帕格治疗的PH患者在FU时风险评估稳定或改善。
