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中华妇幼临床医学杂志(电子版) ›› 2017, Vol. 13 ›› Issue (04) : 378 -383. doi: 10.3877/cma.j.issn.1673-5250.2017.04.002

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儿童系统性红斑狼疮诊断与治疗研究现状
顾珺1, 沈朝斌2,()   
  1. 1. 200080 上海交通大学附属第一人民医院儿科
    2. 200082 上海市中医药大学附属上海市中西医结合医院儿科
  • 收稿日期:2017-03-20 修回日期:2017-06-30 出版日期:2017-08-01
  • 通信作者: 沈朝斌

Research update on diagnosis and treatment of pediatric systemic lupus erythmatosus

Jun Gu1, Chaobin Shen2,()   

  1. 1. Department of Pediatrics, Shanghai General Hospital, Shanghai 200080, China
    2. Department of Pediatrics, Shanghai TCM-Integrated Hospital, Shanghai University of TCM, Shanghai 200082, China
  • Received:2017-03-20 Revised:2017-06-30 Published:2017-08-01
  • Corresponding author: Chaobin Shen
  • About author:
    Corresponding author: Shen Chaobin, Email:
引用本文:

顾珺, 沈朝斌. 儿童系统性红斑狼疮诊断与治疗研究现状[J]. 中华妇幼临床医学杂志(电子版), 2017, 13(04): 378-383.

Jun Gu, Chaobin Shen. Research update on diagnosis and treatment of pediatric systemic lupus erythmatosus[J]. Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition), 2017, 13(04): 378-383.

系统性红斑狼疮(SLE)是一种慢性、多系统损害的自身免疫性疾病。儿童期SLE的发病率及其导致的患儿病死率,均较成年人SLE的发病率及其导致的患者病死率高。儿童SLE早期发病与遗传易感等位基因突变密切相关。2012年,系统性红斑狼疮国际合作组织(SLICC)发布的SLE临床诊断标准,提高了临床诊断该病的准确率。临床对于儿童SLE早期采用免疫抑制治疗的目的,在于短时间内抑制患儿自身免疫反应和非特异性炎症,降低SLE的活动性。笔者拟聚焦儿童SLE的诊断和治疗最新研究进展进行阐述。

Systemic lupus erythematosus (SLE) is a kind of disease with chronic multisystem autoimmune disorders. Childhood-onset SLE is associated with higher morbidity and mortality than those of adult-onset SLE. Early onset of SLE is associated with an increased number of known SLE-susceptibility risk alleles. The Systemic Lupus International Collaborating Clinics (SLICC) developed SLE clinical diagnosis criteria in 2012, which exhibited a higher sensitivity and lower specificity than other criteria before. The aim of child SLE treatment with early immunosuppression is to inhibit autoimmune response and non-specific inflammation in a short time and decrease SLE activity. This article tries to elaborates current diagnosis and treatment of SLE in children.

表1 SLICC制定的SLE临床诊断标准
临床分类指标 免疫学指标
急性皮肤红斑:面部蝶形红斑、大泡性狼疮、中毒性表皮坏死松解症、斑丘疹和光敏性皮炎,并排除皮肌炎和亚急性皮肤狼疮 抗核抗体呈阳性
慢性皮肤狼疮:典型盘状狼疮、局部或广泛的增厚性狼疮、狼疮性脂膜炎、黏膜狼疮、全身性狼疮、冻疮样狼疮和苔藓样重叠样狼疮 抗双链DNA抗体呈阳性,ELISA法检测抗双链DNA抗体滴度结果显示≥2倍滴度
口腔溃疡:无其他病因可解释的上腭、颊部、舌体或鼻黏膜溃疡 抗sm抗体呈阳性
非瘢痕性脱发:无其他病因可解释的毛发弥漫性稀薄或发质脆性增加,抗磷脂抗体呈阳性:包括抗狼疮凝集物,RPR结果呈假阳性,抗心磷脂抗体中等或高度滴度增高(IgA、IgG或IgM),抗β2-糖蛋白滴度增高(IgA、IgG或IgM) ?
≥2个关节发生滑膜炎,表现为关节肿胀和关节液渗出,关节晨僵症状持续30 min以上 补体水平降低:补体3、4水平降低,总补体活性降低
典型的胸、肋膜浆膜炎,或胸腔积液、胸膜摩擦音、心包炎样疼痛≥1 d,或心电图检查结果呈心包炎样 无溶血性贫血时,直接Coombs试验结果呈阳性
肾脏:尿蛋白/肌酐水平增高,或24 h尿蛋白水平>500 mg,可有红细胞管型出现 ?
神经系统:无其他病因可解释的惊厥、神经精神症状、多发性单神经炎、脊髓炎、外周或颅内神经病变和急性意识丧失等 ?
溶血性贫血 ?
白细胞计数降低:无其他病因可解释的白细胞计数<4×109/L(>1次) ?
血小板计数降低:无其他病因可解释的血小板计数<100×109/L(>1次) ?
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