摘要
目的探讨抗磷脂综合征(antiphospholipid syndrome,APS)并发颅内静脉窦血栓(cerebral venous sinus thrombosis,CVST)的临床特点,筛查CVST可能高危因素。方法回顾性分析北京协和医院2004年1月至2014年12月期间住院治疗APS患者中并发CVST的临床表现,实验室及影像学检查特点、血栓形成部位以及治疗和结局。结果 APS并发CVST患者14例,女性8例,中位年龄为34.5(22.0,48.5)岁,中位病程3.5(0.9,10.3)个月。12例(85.7%)出现头痛,6例(42.9%)在病程中曾出现意识障碍,8例(57.1%)出现视盘水肿。磁共振静脉血管成像结果显示横窦受累13例(92.9%),乙状窦9例(64.3%),上矢状窦6例(42.9%),下矢状窦4例(28.6%)。APS并发CVST患者补体降低发生率明显高于APS未并发CVST者,差异有统计学意义(57.1%vs.19.0%,P=0.006)。所有患者均接受低分子肝素抗凝、脱水降颅压治疗,2例接受静脉窦内尿激酶溶栓治疗,13例患者接受激素及免疫抑制剂治疗。1例放弃出院,13例患者病情改善,未再出现病情复发。结论 CVST是APS少见中枢神经系统并发症之一,早期诊断难度大,对于无明确诱因的CVST患者需要警惕APS可能性。APS患者出现颅高压相关症状时,应尽早完善头颅影像学评估CVST,做到早期诊断、早期治疗,以改善预后。
Objective To investigate the clinical and neuroimaging characteristics of antiphopholipid syndrome（ APS） patients complicated with cerebral venous sinus thrombosis（ CVST）. Methods Patients with APSCVST admitted to Peking Union Medical College Hospital from January 2004 to December 2014 were enrolled and analyzed retrospectively. Demographic data, age at onset, disease duration, clinical manifestations, imaging characteristics, management and prognosis were collected. Results Fourteen patients were included,in which 8 were female. The median age was 34. 5（ 22. 0,48. 5）,and the median disease duration was 3. 5（ 0. 9, 10. 3）. Headache（ 85. 7%） was the most common neurological symptoms,followed by altered mental status（ 42. 9%）. Compared with non-CVST APS patients,APSCVST patients had significantly more frequent of hypocomplementemia（ 57. 1% vs. 19. 0%,P = 0. 006）.Magnetic resonance venography（ MRV） showed that common locations of thrombosis were transverse sinus（ 92. 9%）, sigmoid sinus（ 64. 3%）, superior sagittal sinus（ 42. 9%）,and inferior sagittal sinus（ 28. 6%）. All patients were treated with anticoagulant therapy,two patients were treated with local intravenous thrombolysis. Conclusion Patients with CVST without definite primary hyper-coagulation status should routinely be tested for antiphospholipid antibodies. Aggressive treatment for the underlying diseases combined with anticoagulant therapy may improve the prognosis of APS patients complicated with CVST.
出处
《中华临床免疫和变态反应杂志》
2016年第1期4-9,共6页
Chinese Journal of Allergy & Clinical Immunology
基金
国家高技术研究发展计划(863计划)(2012AA02A513)
关键词
抗磷脂综合征
颅内静脉窦血栓
颅内压增高
磁共振静脉成像
狼疮抗凝物
抗凝
antiphopholipid syndrome
cerebral venous sinus thrombosis
intracranial hypertension
magnetic resonance venography
lupus anticoagulant
anticoagulation
