神经精神狼疮的诊断和治疗  

Neuropsychiatric lupus:diagnosis and treatment

作　　者：王元[1] 顾越英[1] 鲍春德[1] 陈顺乐[1] 周滟[2] 许建荣[2] 钱可久[3] 

机构地区：[1]上海第二医科大学附属仁济医院风湿病科 [2]上海第二医科大学附属仁济医院放射科 [3]上海第二医科大学附属仁济医院神经内科

出　　处：《中华风湿病学杂志》2004年第4期230-233,共4页Chinese Journal of Rheumatology

摘　　要：目的对神经精神狼疮患者的诊断和治疗进行临床总结.方法对83例SLE患者进行回顾性分析总结.42例为神经精神狼疮(NPSLE)患者;41例为无临床神经精神症状的SLE对照组.对两组病人实验室检查,包括血液:细胞计数、电解质、肝肾功能、血糖、血脂、血沉;免疫学检测:补体、抗dsDNA抗体、可提取的核抗原(ENA)、间接荧光抗核抗体(IFANA)、抗磷脂抗体等;尿:尿常规、24 h尿蛋白;脑脊液:常规、生化、脑脊液压力、细菌、霉菌学检查、免疫球蛋白检测;影像学检查:脑电图(EEG)、脑CT、脑核磁共振.治疗:对于昏迷、癫痫大发作、急性意识障碍的重症NPSLE患者治疗的甲泼尼龙(MP)起始剂量约200～500 mg/d;对于一般认知障碍、磷脂抗体综合征、局灶性NPSLE(FNPSLE)的泼尼松剂量约1～2mg·kg-1·d-1.当MP剂量渐减至100 mg/d应及时适当应用环磷酰胺(CTX)、甲氨蝶呤(MTX)、硫唑嘌呤(AZA)等免疫抑制剂.统计学分析:Fisher′s精确试验和t检验.结果临床症状方面,NPSLE和SLE对照组比较,面部红斑(71%:44%)和皮肤/胃肠道血管炎的发生率(67%:27%)差异有显著性(P＜0.001).血清学检测,两组比较,仅抗磷脂抗体(aCL)和/或抗β2糖蛋白1(β2GP1)差异有显著性,其发生率分别为43%和22%(P＜0.05).42例NPSLE患者,其中33例为弥漫性NPSLE(DNPSLE),9例为FNPSLE.该两组比较,DNPSLE患者的蛋白尿和皮肤/胃肠道血管炎的发生率显著高于FNPSLE(P＜0.001).42例NPSLE患者,4例死亡;38例NPSLE治疗前后的SLEDAI积分分别为19.71和6.47,经统计分析差异有非常显著性(P＜0.001).结论①NPSLE的确诊必须排除感染、电解质等其他原因所致的SLE中枢神经系统损害;②结合CSF、EEG、脑MRI分析可提高NPSLE诊断的敏感性;③对重症NPSLE患者,静脉滴注MP 500 mg/d×3 d是有益的,对临床和MRI呈现轻度至中等度损害的NPSLE患者,基本激素(泼尼松)剂量1～2mg·kg-1·d-1;④APS所致的NPSLE患者,中等剂量的�Objective To summarize the data of diagnosis and treatment of neuropsychiatric disorders in systemic lupus erythematosus.Methods Eighty-three hospitalized patients with SLE admitted between 1996 to 2002,42 patients with neuropsychiatric lupus (NPSLE),41 patients with no clinical neuropsychitric disorders of SLE.Laboratory study:serologic,urinary and CSF analysis were done including cell count,biochemistry,immunology and CSF culture;EEG,CT and MRI of the brain.Treatment:initial MP 200~500 mg/d were used for patients with coma,grand mal and acute confusion state.Prednisone 1~2 mg·kg-1·d-1 for congnitive dysfunction,antiphosphalipid syndrome and focal NPSLE.CTX,AZA and MTX were used when possible.Statistical analysis:Fisher's test and t-test.Results Patients with NPSLE were compared with SLE control.The prevalence of facial rash and dermal/gastrointestinal vasculitis were 71%∶44% and 67%∶27% respectively(P<0.001);the prevalence of anticardiolipin/anti-β2GP1 antibodies were 43%∶22% (P<0.05).Thirty-three cases of NPSLE patients were diffuse NPSLE (DNPSLE),other 9 cases were focal NPSLE(FNPSLE).Compared with FNPSLE ,the prevalence of proteinuria and dermal/gastrointestinal vasculitis in DNPSLE group was significantly higher (P<0.001).Four cases of 42 NPSLE patients died.The SLEDAI before and after treatment of 38 NPSLE patients were 19.71 and 6.47 respectively (P<0.001).Conclusion Combining CSF analysis with cranial MRI and EEG examination can increase the sensitivity of NPSLE diagnosis.For most severe NPSLE cases,pulse intravenous MP 500 mg for 3 days may be beneficial.1~2 mg·kg-1·d-1 prednisone is essential for NPSLE with manifestation of moderate lesion in T2 weighted and flare sequences of MRI.In NPSLE patients with APS,medium dose of prednisone and anticoagulation therapy must be given.

关 键 词：神经精神狼疮 诊断 治疗 系统性红斑狼疮 神经精神症状 实验室检查 

分 类 号：R593.24[医药卫生—内科学]