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肾脏病与透析肾移植杂志 ›› 2018, Vol. 27 ›› Issue (5): 418-423.DOI: 10.3969/j.issn.1006-298X.2018.05.004

• 论文 • 上一篇    下一篇

系统性红斑狼疮相关可逆性后部脑病综合征

  

  • 出版日期:2018-10-28 发布日期:2018-11-14

Characteristics of posterior reversible encephalopathy syndrome associated with systemic lupus erythematosus

  • Online:2018-10-28 Published:2018-11-14

摘要:

目的:分析系统性红斑狼疮(SLE)合并可逆性后部脑病综合征(PRES)的临床特点、治疗及预后。
方法:回顾性分析SLE合并PRES患者的临床表现、实验室检查及影像学资料,探讨其发病机制、危险因素、治疗及预后特点。
结果:本研究共纳入16例SLE患者,女性14例、男性2例,平均年龄(228±85)岁,16例均有狼疮重度活动, SLE活动性指数为(209±37)分。发生PRES前15例(938%)有高血压,12例(75%)合并肾功能不全、淋巴细胞计数低下、高脂血症,16例患者均使用激素或免疫抑制剂。临床症状有癫痫发作(16例,100%)、头痛(5例,31%)、视觉障碍(5例,31%),恶心呕吐(6例,38%)。CT及磁共振成像(MRI)显示病灶多位于双侧顶枕叶,也可累及颞叶、额叶、脑室、基底节、小脑及皮层。降压、抗癫痫和小剂量激素控制狼疮活动后症状可缓解。
结论:SLE合并PRES是一种临床影像综合征,发病与高血压、肾功能不全、狼疮重度活动等因素相关,临床表现有头痛、视力障碍、癫痫发作和恶心呕吐,脑部影像学表现为双侧顶枕区的皮层下血管源性水肿。SLE合并PRES时若能及时诊断并治疗,病变可逆,预后良好。

关键词: 系统性红斑狼疮, 可逆性后部脑病综合征, 影像学

Abstract:

Objective:To explore the clinical features,treatment and prognosis of systemic lupus erythematosus (SLE) patients with posterior reversible encephalopathy syndrome (PRES).
Methodology:Sixteen SLE patients with PRES were enrolled into this retrospective studyTheir pathogenesis,risk factors,treatment and prognosis were investigated.
Results:They were 14 female and 2 male with a mean age of (228±85) years old(ranged from 9 to 43).All 16 cases had high systemic activity,the mean SLEDAI score was (209±37).15 cases(938%) had hypertension,12 cases(75%) had renal insufficiency,low lymphocyte count,dyslipidemia,and all of cases(100%) used hormones or immunosuppressants.Clinical manifestations included seizures in 16 (100%),headache in 5 (31%),visual disturbances in 5 (31%),and nausea and vomiting in 6 (38%).CT and magnetic resonance imaging (MRI) showed that the lesions were mostly located in bilateral parietooccipital lobes.Temporal lobes,frontal lobes,ventricles,basal ganglia,cerebellum,and cortex may also be involved in.Antihypertensive,antiepileptic and lowdose glucocorticoids  relieve symptoms.
Conclusion:SLE patients with PRES is a clinicalimaging syndrome,which often associated with hypertension,renal insufficiency and systemic disease activity.Clinical manifestations include headache,visual disturbances,seizures,nausea and vomiting.Brain imaging manifests as subcortical vasogenic edema in the bilateral parietooccipital area.Early diagnosis and treatment generally predicates good prognosis.

Key words: systemic lupus erythematosus, posterior reversible encephalopathy syndrome, image