M. Di Battista, MD, Rheumatology Unit, University of Pisa, Pisa, and Department of Medical Biotechnologies, University of Siena, Siena;
A. Della Rossa, MD, PhD, M. Da Rio, MD, G. De Mattia, MD, M. Mosca, MD, PhD, Rheumatology Unit, University of Pisa, Pisa.
J Rheumatol. 2023 Aug;50(8):1029-1031. doi: 10.3899/jrheum.221113. Epub 2023 Apr 1.
Systemic sclerosis (SSc) is burdened by Raynaud phenomenon (RP) and digital ulcers (DUs), and sometimes standard vasoactive therapies are ineffective or contraindicated. Selexipag is an oral selective IP prostacyclin receptor agonist approved for the treatment of SSc-related pulmonary arterial hypertension. We aimed to evaluate the clinical and instrumental efficacy of selexipag in SSc digital vasculopathy.
Patients with SSc with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies were administered selexipag. RP- and DU-related clinical outcomes were evaluated, and digital perfusion was assessed by laser speckle contrast analysis (LASCA), all at baseline and after 3 months.
Selexipag was administered to 9 patients with SSc (66.6% female, mean age 52.3 [SD 16.6] yrs). One patient had to stop the drug because of adverse effects. After 3 months of selexipag administration, there was a significant reduction in RP daily episodes ( = 0.01) and RP mean duration ( = 0.04). The number of DUs decreased from 10 to 4 without reaching statistical significance. A significant improvement in mean perfusion of the fingers ( = 0.02) was observed with LASCA.
Selexipag showed good potential for the treatment of SSc digital vasculopathy. Our results are certainly preliminary, yet quite encouraging. New trials for the evaluation of selexipag efficacy in SSc digital vasculopathy are needed.
系统性硬化症（SSc）伴有雷诺现象（RP）和指溃疡（DU），有时标准的血管活性治疗无效或禁忌。塞来昔帕是一种口服选择性 IP 前列环素受体激动剂，已被批准用于治疗 SSc 相关肺动脉高压。我们旨在评估塞来昔帕在 SSc 手指血管病变中的临床和仪器疗效。
患有严重手指血管病变的 SSc 患者，对所有其他血管活性治疗均无反应或禁忌，给予塞来昔帕。在基线和 3 个月时，评估 RP 和 DU 相关的临床结局，并通过激光散斑对比分析（LASCA）评估手指灌注。
9 例 SSc 患者（66.6%为女性，平均年龄 52.3[16.6]岁）接受了塞来昔帕治疗。1 例患者因不良反应停止用药。塞来昔帕治疗 3 个月后，RP 每日发作次数（ = 0.01）和 RP 平均持续时间（ = 0.04）显著减少。DU 数量从 10 个减少到 4 个，但无统计学意义。LASCA 观察到手指平均灌注明显改善（ = 0.02）。
塞来昔帕在治疗 SSc 手指血管病变方面显示出良好的潜力。我们的结果当然是初步的，但非常令人鼓舞。需要新的试验来评估塞来昔帕在 SSc 手指血管病变中的疗效。
