Abstract：
BACKGROUND: Sneddon syndrome is an occlusive vasculopathy that presents clinically with generalized livedo racemosa on the skin and transient ischemic attacks, strokes, and cognitive or motor deficits in the central nervous system. Antiplatelet or anticoagulant therapy is recommended. Due to the limited therapeutic efficacy and the resulting serious complications, we propose combination therapy with additional infusion cycles of alprostadil and captopril and report initial long-term results.
METHODS: We performed a systematic retrospective analysis of all patients with primary Sneddon syndrome who received combination therapy in our clinic between 1995 and 2020. Therapeutic outcomes were evaluated using descriptive statistics compared to historical controls receiving monotherapy. We also analyzed the event rate of complications when combination therapy was discontinued.
RESULTS: During the 99.7 patient-years of follow-up, there were no transient ischemic attacks and the stroke rate dropped to 0.02 per patient-year. In comparison, the rates of transient ischemic attacks and strokes in the historical controls ranged from 0.08 to 0.035 per patient-year. After discontinuation of alprostadil therapy, eight events occurred in three patients.
CONCLUSIONS: Combination therapy reduces the long-term incidence of ischemic events in patients with primary Sneddon syndrome.
METHODS: We performed a systematic retrospective analysis of all patients with primary Sneddon syndrome who received combination therapy in our clinic between 1995 and 2020. Therapeutic outcomes were evaluated using descriptive statistics compared to historical controls receiving monotherapy. We also analyzed the event rate of complications when combination therapy was discontinued.
RESULTS: During the 99.7 patient-years of follow-up, there were no transient ischemic attacks and the stroke rate dropped to 0.02 per patient-year. In comparison, the rates of transient ischemic attacks and strokes in the historical controls ranged from 0.08 to 0.035 per patient-year. After discontinuation of alprostadil therapy, eight events occurred in three patients.
CONCLUSIONS: Combination therapy reduces the long-term incidence of ischemic events in patients with primary Sneddon syndrome.
摘要：
背景：Sneddon综合征是一种闭塞性血管病变，临床上表现为皮肤上的全身性Livedoracemosa和短暂性脑缺血发作，笔画,和中枢神经系统的认知或运动缺陷。推荐抗血小板或抗凝治疗。由于治疗效果有限及由此产生的严重并发症,我们建议联合使用前列地尔和卡托普利额外输注周期,并报告初步长期结果.
方法：我们对1995年至2020年在我们诊所接受联合治疗的所有原发性Sneddon综合征患者进行了系统的回顾性分析。与接受单一疗法的历史对照相比，使用描述性统计来评估治疗结果。我们还分析了停止联合治疗时并发症的事件发生率。
结果：在99.7患者年的随访中，无短暂性脑缺血发作,卒中发生率降至0.02/患者-年.相比之下,在历史对照中,短暂性脑缺血发作和卒中的发生率为0.08~0.035/患者-年.停止前列地尔治疗后，3例患者发生8例事件.
结论：联合治疗可降低原发性Sneddon综合征患者缺血事件的长期发生率。
方法：我们对1995年至2020年在我们诊所接受联合治疗的所有原发性Sneddon综合征患者进行了系统的回顾性分析。与接受单一疗法的历史对照相比，使用描述性统计来评估治疗结果。我们还分析了停止联合治疗时并发症的事件发生率。
结果：在99.7患者年的随访中，无短暂性脑缺血发作,卒中发生率降至0.02/患者-年.相比之下,在历史对照中,短暂性脑缺血发作和卒中的发生率为0.08~0.035/患者-年.停止前列地尔治疗后，3例患者发生8例事件.
结论：联合治疗可降低原发性Sneddon综合征患者缺血事件的长期发生率。
