系统性红斑狼疮合并颅内静脉窦血栓的临床特点

目的探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)合并颅内静脉窦血栓(cerebral venous sinus thrombosis,CVST)的临床特点。方法回顾性分析北京协和医院2000年1月至2013年6月SLE中合并CVST患者的神经系统表现、全身病情活动度、血栓形成部位、影像学特点及治疗和预后。结果 SLE并发CVST患者13例,占同期SLE住院患者(4540例)的0.29%。女10例,男3例,平均年龄30.5岁。神经系统表现以头痛(1113)、视力下降(713)、意识障碍(513)、癫痫(413)最为常见。脑脊液压力升高9例,其中7例330 mm H2O,脑脊液蛋白升高5例。磁共振静脉血管成像提示血栓形成部位常见于横窦(1113)、乙状窦(813)、上矢状窦(413)等,多数(1013)患者为2处以上静脉窦血栓形成。患者平均SLE疾病活动指数为(11.7±2.8)分(不包括CVST)。经脱水降颅压、抗凝、糖皮质激素冲击、免疫抑制剂及地塞米松鞘注治疗,9例患者好转,3例死亡,1例失访。结论 CVST是SLE的罕见并发症,多发生于SLE活动期,有特殊的临床症状、体征及脑脊液压力升高等提示,需早期识别、积极治疗以改善预后。

Clinical Characters of Cerebral Venous Sinus Thrombosis in Systemic Lupus Erythematosus

Objective To summarize the clinical characteristics of SLE patients complicated with cerebral venous sinus thrombosis （CVST）.Methods The neurological manifestations,disease activity of SLE,imaging features,treatment and prognosis of hospitalized lupus patients with CVST in Peking Union Medical College Hospital from January 2000 to June 201 3 were retrospectively analyzed.Results Thirteen SLE patients with CVST accounted for 0.29% of the 4540 hospitalized SLE patients.Ten were female and 3 were male, with the mean age of 30.5 year.Headache （84.6%）was the most common neurological symptom followed by decreased visual acuity （53.8%）,unconsciousness （38.5%）and epilepsy （30.8%）.Increased intracranial pressure was found in 9 cases and that of 7 of them was even higher than 330 mm H2 O.CSF protein level was elevated in 5 cases.Magnetic resonance venography （MRV）showed that the common locations of thrombosis were the transverse sinus （1 1 /1 3）,sigmoid sinus （8/1 3）and the vector sinus （4/1 3）;the majority （1 0/1 3）had two or more locations of venous sinus thrombosis.CVST often occurred in active SLE with the average SLE disease activity index （SLEDAI ） score of （1 1 .7 ±2.8 ）（CVST excluded）.Treated by corticosteroid and immunosuppressants including cyclophosphamide,as well as intrathecal dexamethasone injection, anticoagulants and dehydration, 9 patients achieved clinical improvements,3 patients died at hospital and,the other 1 was lost during follow-up.Conclusions The incidence of CVST in SLE patients is relatively rare.CVST tends to occur in patients with active lupus. Certain clinical symptoms,signs,and elevated intractanial pressures should be identified early and paid more attention should be paid to this rare manifestation.Aggressive treatment for the underlying diseases combined with anticoagulant therapy may improve the prognosis of SLE patients complicated with CVST.