儿童期起病的系统性红斑狼疮神经精神综合征的发病率和患病率

The incidence and prevalence of neuropsychiatric syndromes in pediatric onset systemic lupus erythematosus.

作者信息

Sibbitt Wilmer L, Brandt John R, Johnson Courtney R, Maldonado Marcos E, Patel Samir R, Ford Corey C, Bankhurst Arthur D, Brooks William M

机构信息

Clinical and Magnetic Resonance Research Center, Department of Internal Medicine, The University of New Mexico Health Sciences Center, Albuquerque 87131, USA.

出版信息

J Rheumatol. 2002 Jul;29(7):1536-42.

PMID:12136916

Abstract

OBJECTIVE

To determine the incidence and prevalence of neuropsychiatric systemic lupus erythematosus (NPSLE) and glomerulonephritis in ethnically diverse pediatric onset SLE inpatient and outpatient populations.

METHODS

Seventy-five pediatric onset patients with SLE including Native American, Asian, Black, Spanish-American, and Caucasian subjects were evaluated prospectively and cross sectionally. During the 6 year study, 55 patients became inpatients. Subjects underwent medical interview, physical examination, laboratory review, neuropsychiatric inventory, and chart review. Classification of NPSLE was accomplished with the 1999 ACR NPSLE case definitions.

RESULTS

Prospectively, NPSLE occurred in 95% of pediatric SLE patients and was more common than glomerulonephritis (55%; p < or = 0.0001). NPSLE prevalence (%) and incidence (event/person/yr) were as follows: headache 72%, 95; mood disorder 57%, 0.41; cognitive disorder 55%, 0.49; seizure disorder 51%, 0.94; acute confusional state 35%, 0.6; anxiety disorder 21%, 0.28; peripheral nervous system disorder 15%, 0.16; cerebrovascular disease 12%, 0.32; psychosis 12%, 0.16; chorea 7%, 0.01; demyelinating syndrome 4%, 0.01; and myelopathy 1%, 0.001. Cross sectionally, active NPSLE was present in 93% of inpatients and 69% of outpatients. When only serious forms of NPSLE were considered (stroke, seizures, major cognitive disorder, chorea, psychosis, major depression, acute confusional state), serious or life-threatening NPSLE occurred in 76% of all SLE subjects prospectively, and in 85% and 40% of inpatients and outpatients cross sectionally, which in each instance was more common than glomerulonephritis (p < or = 0.001).

CONCLUSION

NPSLE is one of the most common serious complications of pediatric SLE, and is particularly increased in pediatric inpatients.

摘要

目的

确定不同种族儿童期起病的系统性红斑狼疮（SLE）住院和门诊患者中神经精神性系统性红斑狼疮（NPSLE）和肾小球肾炎的发病率及患病率。

方法

对75例儿童期起病的SLE患者进行前瞻性和横断面评估，这些患者包括美国原住民、亚洲人、黑人、西班牙裔美国人和白人。在为期6年的研究中，55例患者成为住院患者。研究对象接受了医学访谈、体格检查、实验室检查、神经精神量表评估及病历审查。NPSLE的分类依据1999年美国风湿病学会（ACR）NPSLE病例定义。

结果

前瞻性研究中，95%的儿童SLE患者发生NPSLE，其比肾小球肾炎更常见（55%；p≤0.0001）。NPSLE的患病率（%）及发病率（事件/人/年）如下：头痛72%，0.95；情绪障碍57%，0.41；认知障碍55%，0.49；癫痫障碍51%，0.94；急性意识模糊状态35%，0.6；焦虑障碍21%，0.28；周围神经系统障碍15%，0.16；脑血管疾病12%，0.32；精神病12%，0.16；舞蹈病7%，0.01；脱髓鞘综合征4%，0.01；脊髓病1%，0.001。横断面研究中，93%的住院患者和69%的门诊患者存在活动性NPSLE。仅考虑严重形式的NPSLE（中风、癫痫发作、重度认知障碍、舞蹈病、精神病、重度抑郁、急性意识模糊状态）时，前瞻性研究中76%的SLE患者发生严重或危及生命的NPSLE，横断面研究中住院患者和门诊患者的这一比例分别为85%和40%，且在每种情况下均比肾小球肾炎更常见（p≤0.001）。