儿童神经精神狼疮22例临床特征与远期预后分析  

Clinical features and long-term prognosis analysis of 22 children with neuropsychiatric lupus erythematosus

作　　者：徐海霞 胡斌 郑琪[1] 杨兴惠[2] 卢美萍[1] Xu Haixia;Hu Bin;Zheng Qi;Yang Xinghui;Lu Meiping(Department of Rheumatology Immunology and Allergy,the Children′s Hospital of Zhejiang University school of Medicine,Hangzhou 310000,China;Department of Radiology,the Children′s Hospital of Zhejiang University school of Medicine,Hangzhou 310000,China;Department of Rheumatology Immunology,Guiyang Maternal&Child Health Care Hospital,Guiyang Children′s Hospital,Guiyang 550003,China)

机构地区：[1]浙江大学医学院附属儿童医院风湿免疫过敏科,杭州310000 [2]浙江大学医学院附属儿童医院放射科,杭州310000 [3]贵阳市妇幼保健院(贵阳市儿童医院)风湿免疫科,贵阳550003

出　　处：《中华风湿病学杂志》2024年第11期789-795,共7页Chinese Journal of Rheumatology

基　　金：国家自然科学基金(82070027);浙江省“尖兵”“领雁”研发攻关计划资助(2023C03032)。

摘　　要：目的通过分析神经精神狼疮(NPSLE)患儿的临床特征及预后,为该病的诊治提供参考。方法回顾性总结分析2019年1月至2022年3月浙江大学医学院附属儿童医院风湿免疫过敏科收治的22例NPSLE患儿的临床资料,并进行24~60个月的随访。采用SPSS 23.0统计软件进行统计学描述。结果22例NPSLE患儿占同期系统性红斑狼疮(SLE)住院患儿的26.8%(22/82),男∶女=1∶4.5,发病年龄(10.7±2.0)岁。86.4%(19/22)为初发且呈重度疾病活动度,16例(72.7%)发生于病程1个月内。合并皮肤黏膜损害19例(86.4%)、狼疮肾炎16例(72.7%)、血液系统损害14例(63.2%)、骨骼肌肉损害13例(59.1%)、浆膜炎及高血压各10例(45.5%)、巨噬细胞活化综合征(MAS)8例(36.5%)。神经系统临床表现依次为头痛(11/19,57.9%)、头晕(10/19,52.6%)、精神萎靡(7/19,36.8%)、视物模糊(4/19,21.1%)、抽搐(3/19,15.8%)、嗜睡(2/19,10.5%)。头颅MRI异常20例(20/22,90.9%),头颅CT异常7例(7/12,58.3%),脑电图异常10例(10/22,45.5%),脑脊液检查异常6例(6/20,30.0%)。随访34(28,48)个月,SLE临床缓解或低疾病活动度19例(86.4%);除3例残留脑梗死病灶,所有患儿均未遗留神经系统后遗症,无死亡病例。结论儿童NPSLE最常见的症状是头痛、头晕,好发于初发且呈重度疾病活动度患者,约36.5%可能合并MAS。24~60个月随访结果显示本病预后良好。Objective To analyze the clinical features and follow-up data in children with neuropsychiatric lupus erythematosus(NPSLE),and provide reference for the diagnosis and treatment of NPSLE.Methods The clinical data of 22 children with NPSLE who were admitted to the Department of Rheumatology Immunology and Allergy,Children′s Hospital of Zhejiang University School of Medicine from January 2019 to March 2022 were included and were followed-up for 24~60 months.The data were analyzed retrospectively.Statistical descriptive analysis was performed using the SPSS 23.0.Results Twenty-two(26.8%,22/82)children with NPSLE occurred in the hospitalized children with SLE during the study period.The ratio of male to female was 1∶4.5,and the onset age was(10.7±2.0)years.Among these cases,86.4%(19/22)patients were newly diagnosed and had severe disease activity and 16 cases(72.7%)occurred within 1 month after disease onset.Nineteen cases(86.4%)had mucocutaneous involvement,16 cases(72.7%)had lupus nephritis,14 cases(63.2%)had hematological system involvement,13 cases(59.1%)had skeletal muscle involvement,10 cases(45.5%)had serositis,10 cases(45.5%)were complicated with hypertension,and 8 cases(36.5%)with macrophage activation syndrome(MAS).The main clinical symptoms of the nervous system included headache(11/19,57.9%),dizziness(10/19,52.6%),listlessness(7/19,36.8%),blurred vision(4/19,21.1%),convulsions(3/19,15.8%),lethargy(2/19,10.5%).Twenty patients(20/22,90.9%)demonstrated abnormal signals on brain MRI,7 cases(7/12,45.5%)showed abnormal signals on brain CT,10 cases(10/22,45.5%)showed abnormal waves on EEG,and 6 cases(6/20,30.0%)demonstrated abnormal results of cerebrospinal fluids analysis.The follow-up duration was 34(28,48)months.Clinical remission or low disease activity was found in 19 patients(86.4%),and no death were observed.Three cases had residual cerebral infarction lesions,no neurological sequelae were found in all patients.Conclusion The most common symptoms of NPSLE in children are headache and dizziness,which a

关 键 词：红斑狼疮 系统性 狼疮血管炎 中枢神经系统 巨噬细胞活化综合征 儿童 

分 类 号：R725.9[医药卫生—儿科]