Department of Neurology, Faculty of Medicine, Juntendo University, Tokyo, Japan.
Department of Neurosurgery, Faculty of Medicine, Juntendo University, Tokyo, Japan.
Lupus. 2021 Jan;30(1):118-124. doi: 10.1177/0961203320960821. Epub 2020 Oct 4.
Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by thromboembolic events, including ischemic stroke or complications in pregnancy, and the presence of antiphospholipid antibodies. Cervical artery dissection (CAD) is not an uncommon cause of stroke in young adults. The concomitant presence of APS and CAD is extremely rare.
Two cases with APS who developed acute ischemic strokes related to CAD are reported. A comprehensive systematic literature search using the PubMed database was also conducted.
In Case 1, a 36-year-old woman who had been diagnosed with systemic lupus erythematosus and had been repeatedly positive for lupus anticoagulant tests developed an ischemic stroke caused by a vertebral artery dissection (VAD). After admission, she had a recurrent ischemic stroke, followed by considerable changes in steno-occlusive lesions of the vertebrobasilar artery system. In Case 2, a 36-year-old man developed multiple brain infarcts due to bilateral VAD with aneurysmal formations and associated with pulmonary embolism. The anticardiolipin antibody titer was repeatedly elevated after stroke. The literature review identified 8 patients with CAD associated with APS, involving the internal carotid artery in 6 patients and the middle cerebral artery and vertebral artery in 1 patient each. The patients were predominantly young and female, infrequently had atherosclerotic vascular risk factors, and were positive for various antiphospholipid antibodies.
The current report described two rare cases of ischemic stroke caused by CAD secondary to APS, along with a review of the literature; the patients displayed characteristic clinical manifestations, implying specific mechanisms for cerebral artery disorders secondary to APS.
抗磷脂综合征（APS）是一种自身免疫性疾病，其特征为血栓栓塞事件，包括缺血性中风或妊娠并发症，以及存在抗磷脂抗体。颈动脉夹层（CAD）并非年轻人中风的常见原因。同时存在 APS 和 CAD 的情况极为罕见。
报告了两例因 CAD 导致急性缺血性中风的 APS 患者。还使用 PubMed 数据库进行了全面的系统文献检索。
在病例 1 中，一名 36 岁女性患有系统性红斑狼疮，狼疮抗凝试验反复阳性，发生了由椎动脉夹层（VAD）引起的缺血性中风。入院后，她发生了复发性缺血性中风，随后椎基底动脉系统的狭窄闭塞性病变发生了重大变化。在病例 2 中，一名 36 岁男性因双侧 VAD 导致多发性脑梗死，伴有动脉瘤形成和肺栓塞。中风后抗心磷脂抗体滴度反复升高。文献复习发现了 8 例与 APS 相关的 CAD，涉及 6 例颈内动脉和 1 例大脑中动脉和椎动脉。患者主要为年轻女性，很少有动脉粥样硬化血管危险因素，且存在各种抗磷脂抗体。
本报告描述了两例罕见的由 APS 继发 CAD 引起的缺血性中风病例，并对文献进行了复习；患者表现出特征性的临床表现，提示 APS 继发的脑动脉疾病具有特定的机制。
