77例神经精神狼疮患儿临床分析  

Clinical manifestations, laboratory test, treatment and outcome of neuropsychiatric involvement inpediatric systemic lupus erythematosus patients

作　　者：吴晓燕[1] 宋红梅[1] 曾小峰[2] 何艳燕[1] 王薇[1] 李凡[1] 郭异珍[1] 魏珉[1] 

机构地区：[1]中国医学科学院中国协和医科大学北京协和医院儿科,北京100730 [2]中国医学科学院中国协和医科大学北京协和医院免疫科,北京100730

出　　处：《中华儿科杂志》2009年第4期286-290,共5页Chinese Journal of Pediatrics

基　　金：国家十一五科技支撑计划资助项目（2008BAI59B00,2008BAI59B02）

摘　　要：目的分析神经精神狼疮（NPSLE）患儿的临床特点。方法回顾性分析77例NPSLE患儿的临床表现、实验室检查、神经影像学特征、治疗及预后，并采用Logistic回归模型分析相关指标与NPSLE复发的关系。结果本组系统性红斑狼疮（SLE）患儿NPSLE患病率为17．3％，75％患儿发病后2年以内出现NPSLE。其表现形式多种多样，最常见的症状是头痛（31．8％），其次是癫痫样发作（29．1％）。活动期92．2％患儿SLE疾病活动指数（SLEDAI）评分≥15分，表现为重症狼疮。常伴有发热（88．3％）、皮疹（84．4％），最易同时受累的系统包括肾脏（76．6％）、血液系统（67．5％）。与其他实验室指标相比，NPSLE急性期抗核抗体（ANA）阳性率最高（98．7％），其次是血沉增快（86．3％）和补体降低（72．7％）。90．1％腰椎穿刺结果异常，60．7％头颅CT异常，54．8％头颅磁共振成像异常，73．9％脑电图异常。常规治疗采用糖皮质激素加免疫抑制剂治疗，79．2％联合应用大剂量甲泼尼龙冲击治疗；51．9％疾病急性期进行了甲氨蝶呤＋地塞米松联合鞘内注射治疗；26．0％急性期应用了静脉免疫球蛋白治疗；2例患儿进行了自体外周血干细胞移植治疗。住院死亡率9．0％，NPSLE复发率22．0％，75．0％患儿24个月内复发。SLEDAI评分对预测NPSLE复发有显著意义（X^2=3．987，P：0．0459，OR=1．172，95％CI1．003-1．370）。结论本研究初步探讨了儿童NPSLE患者的临床特点，分析了可能导致NPSLE复发的相关因素，为进一步认识及合理治疗儿童NPSLE奠定了基础。Objective To investigate the clinical manifestations, laboratory test, treatment andoutcome of neuropsychiatric （NP） involvement in pediatric systemic lupus erythematosus （SLE） patients. Methods Seventy-seven patients with NP syndromes of SLE （NPSLE） seen from 1987 to 2007 were retrospectively reviewed. The relationship between the relative factors and the relapse of NPSLE was analyzed with logistic regression model. Results NPSLE was found in 17. 3% of the SLE patients and 75% of the NPSLE patients the NP involvements occurred in the first 2 years of the onset of SLE. The most frequent NP manifestations were headache （31.8%） and seizure disorder （29.1%）. In the active phases, the levels of Systemic Lupus Erythematosus Disease Activity Index （SLEDAI） scores of the 92. 2% patients were higher than 15 and belonged to severe lupus. The patients were accompanied frequently with fever （88. 3% ） and rash （84.4%）. The most frequently involved organs were kidney （76. 6% ） and blood system （67.5%）. In the active phases, the ANA was positive （98.7%） , the level of ESR increased （86. 3% ）, the level of complement profile decreased （72. 7% ）. The cerebrospinal fluid （CSF） study, the CT, the MRI and the EEG were abnormal （90. 1%, 60.7%, 54. 8%, 73.9%, respectively）. All the patients received glucocorticoids and imnmnodepressant treatment in which 79. 2% received IV high-dose methylprednisolone （ MP）, 51.9% received intrathecal （IT） methotrexate （ MTX ） and dexamethasone （ DXM ）, 26. 0% received IVIG, 2 patients received autologous peripheral blood stem cell transplantation. The mortality was 9.0%. The rate of relapse was 22. 0% and in 75.0% of relapsed patients the relapse occurred within 24 months from the onset of NPSLE. The SLEDAI scores related to the relapse of the NPSLE （ X^2 = 3. 987,P=0. 0459,0R =1. 172,95% CI 1.003 and 1.370）. Conclusion SLEDAI scores were significantly helpful in predicting recurrence of NPSLE.

关 键 词：狼疮血管炎 中枢神经系统 儿童 治疗 临床研究性 

分 类 号：R686[医药卫生—骨科学]