Primary antiphospholipid syndrome complicated with moyamoya syndrome: a case report and literature review
Received date: 2022-01-26
Online published: 2022-11-07
目的：分析1例原发性抗磷脂综合征（primary antiphospholipid syndrome,PAPS）合并烟雾综合征（moyamoya syndrome,MMS）患者的临床资料,并结合文献探讨APS合并MMS的临床特点。方法：收治1例PAPS合并MMS患者的临床资料,并检索国内外相关文献对该病进行分析总结。结果：本例患者为23岁女性,既往有2次病理妊娠史。此次以脑梗死起病,脑血管影像学检查示双侧颈内动脉重度狭窄或闭塞并侧支血管形成,符合MMS表现;腹部CT示脾肿大。患者经抗凝及免疫抑制剂等治疗后症状改善。检索国内外文献,共发现7例有详细临床资料的APS合并烟雾状血管改变患者,均为女性,平均年龄为26.6岁,临床表现异质性大,其中肢体无力是常见症状,临床上以动静脉血栓形成、病理性妊娠及持续性抗磷脂抗体阳性为主要表现,共6例患者进行详细随访,5例患者经抗凝、免疫抑制剂等治疗后症状改善,余1例患者因脑出血死亡。结论：APS为一种累及多系统的自身免疫性疾病,其合并MMS的病例报道非常罕见。APS的抗凝或抗血小板治疗与MMS的侧支血管存在出血风险存在相矛盾,应谨慎选择双重抗血小板治疗。
宋洛卿, 戴廷军 . 原发性抗磷脂综合征合并烟雾综合征一例并文献复习[J]. 诊断学理论与实践, 2022 , 21(04) : 497 -503 . DOI: 10.16150/j.1671-2870.2022.04.013
Objective: To analyze the clinical data of a case of primary antiphospholipid syndrome (PAPS) combined with moyamoya syndrome (MMS), and to explore the clinical characteristics of APS combined with MMS. Methods:The clinical data of a patient with PAPS and MMS were collected and analyzed by searching the relevant literatures. Results: The patient was a 23 year old female with 2 pathological pregnancies. The disease started with cerebral infarction. Cerebrovascular imaging showed severe stenosis or occlusion of bilateral internal carotid arteries with collateral vessels formation, which was consistent with MMS findings. Abdominal CT showed splenomegaly. The symptoms were improved after anticoagulation and immunosuppressive therapy. Through literature review at home and abroad, 7 patients with APS complicated with smoky vascular changes with detailed clinical data were found, all of them were female, with an average age of 26.6 years. Their clinical manifestations were heterogeneous, in which limb weakness was a common symptom, with arteriovenous thrombosis, pathological pregnancy and persistent positive antiphospholipid antibody as the main clinical manifestations. A total of 6 patients were followed up in detail, and the symptoms of the 5 patients were improved after anticoagulation and immunosuppressive therapy. One patient died of cerebral hemorrhage. Conclusions: APS is an autoimmune disease involving multiple systems. The case report of APS with MMS is very rare. The anticoagulant or antiplatelet therapy of APS is contradictory to the risk of bleeding in the collateral vessels of MMS, so dual antiplatelet therapy should be carefully selected.
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