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儿童系统性红斑狼疮中的抗心磷脂抗体

Anticardiolipin antibodies in pediatric systemic lupus erythematosus.

作者信息

Ravelli A, Caporali R, Di Fuccia G, Zonta L, Montecucco C, Martini A

机构信息

Pediatric Clinic, Istituto di Ricovero e Cura a Carattere Scientifico S Matteo, Italy.

出版信息

Arch Pediatr Adolesc Med. 1994 Apr;148(4):398-402. doi: 10.1001/archpedi.1994.02170040064011.

Abstract

OBJECTIVE

To investigate the prevalence and the clinical significance of anticardiolipin antibodies (ACLs) in a group of children with systemic lupus erythematosus (SLE).

DESIGN

Cross-sectional and longitudinal study.

SETTING

Pediatric Clinic, University of Pavia, Italy.

PARTICIPANTS

Thirty children (aged 4.9 to 16.5 years) with SLE.

MEASUREMENTS AND MAIN RESULTS

Twenty-six (87%) of the 30 patients were initially positive for either IgG or IgM ACLs; 24 (80%) of 30 had IgG ACLs, and 15 (50%) of 30 had IgM ACLs. The cross-sectional analysis showed a trend for IgG ACLs to be positively associated with autoimmune cytopenia and negatively associated with renal disease. The levels of ACLs, particularly of the IgG isotype, tended to correlate with SLE activity as expressed by the complement fraction C3, the erythrocyte sedimentation rate, or the SLE Activity Measure, but not by the SLE Disease Activity Index or the anti-DNA antibodies. Serial determinations of ACL levels in 20 patients revealed frequent fluctuations. High levels of IgG ACLs (> 50 arbitrary units) were observed in nine patients; all nine had active disease and eight had one or more clinical features that have been previously associated with antiphospholipid antibodies: neuropsychiatric manifestations in six patients, autoimmune cytopenia in two patients, and avascular necrosis of bone in one patient. Only one patient experienced an overt episode of vascular thrombosis; IgG ACLs were positive at a medium level 6 months before the thrombotic event, but their level was unchanged when the thrombosis was discovered; the lupus anticoagulant test was positive at time of the thrombosis.

CONCLUSIONS

Our results show that in pediatric SLE, ACLs are frequently found, high levels of IgG ACLs are often associated with central nervous system involvement, and ACLs have a low predictive value in the development of vascular thrombosis.

摘要

目的

调查一组系统性红斑狼疮(SLE)患儿中抗心磷脂抗体(ACL)的患病率及其临床意义。

设计

横断面和纵向研究。

地点

意大利帕维亚大学儿科诊所。

参与者

30例SLE患儿(年龄4.9至16.5岁)。

测量指标及主要结果

30例患者中有26例(87%)最初IgG或IgM ACL呈阳性;30例中有24例(80%)IgG ACL阳性,30例中有15例(50%)IgM ACL阳性。横断面分析显示,IgG ACL有与自身免疫性血细胞减少呈正相关、与肾脏疾病呈负相关的趋势。ACL水平,尤其是IgG同种型水平,往往与补体C3、红细胞沉降率或SLE活动度测量所表达的SLE活动相关,但与SLE疾病活动指数或抗DNA抗体无关。对20例患者ACL水平的系列测定显示其频繁波动。9例患者观察到高水平的IgG ACL(>50任意单位);所有9例均有活动性疾病,8例有一项或多项先前与抗磷脂抗体相关的临床特征:6例有神经精神表现,2例有自身免疫性血细胞减少,1例有骨缺血性坏死。仅1例患者发生明显的血管血栓事件;血栓形成事件前6个月IgG ACL呈中等水平阳性,但发现血栓时其水平未变;血栓形成时狼疮抗凝物试验呈阳性。

结论

我们的结果表明,在儿童SLE中,ACL常见,高水平的IgG ACL常与中枢神经系统受累相关,且ACL在血管血栓形成发展中的预测价值较低。

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