系统性红斑狼疮相关可逆性后部脑病综合征  

Characteristics of posterior reversible encephalopathy syndrome associated with systemic lupus erythematosus

作　　者：胡娜娜 董建华 许书添 陈乐波 李世军 HU Nana;DONG Jianhua;XU Shutian;CHEN Lebo;LI Shijun(Bengbu Medical College,Bengbu 233030,China;National Clinical Research Center of Kidney Diseases,Jinling Hosptial,Nanfing University School of Medicine,Nanjing 210016,China)

机构地区：[1]蚌埠医学院,蚌埠233030 [2]南京总医院国家肾脏疾病临床医学研究中心全军肾脏病研究所

出　　处：《肾脏病与透析肾移植杂志》2018年第5期418-423,共6页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家自然科学基金(81270799);江苏省临床医学研究中心项目(YXZX2016003)

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。ABSTRACTObjective：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 immnnosuppressants. 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 treatnlent generally predicates good prognosis.

关 键 词：系统性红斑狼疮 可逆性后部脑病综合征 影像学 

分 类 号：R593.241[医药卫生—内科学]