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儿童和成人系统性红斑狼疮患者之间的异同。

Similarities and differences between pediatric and adult patients with systemic lupus erythematosus.

作者信息

Tarr T, Dérfalvi B, Győri N, Szántó A, Siminszky Z, Malik A, Szabó A J, Szegedi G, Zeher M

机构信息

Department of Clinical Immunology, University of Debrecen, Hungary.

2nd. Department of Pediatrics, Semmelweis University Budapest, Hungary Department of Pediatrics, Dalhousie University-IWK Health Centre, Halifax, Canada.

出版信息

Lupus. 2015 Jul;24(8):796-803. doi: 10.1177/0961203314563817. Epub 2014 Dec 15.

Abstract

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease with highest prevalence among women of childbearing age. However, children younger than 16 years also can develop SLE (childhood-onset lupus/juvenile-type SLE). The aim of our study was to compare the clinical course of adult and pediatric-onset SLE. Data from 342 adult patients followed at the University of Debrecen, Hungary, and 79 children documented in the Hungarian National Pediatric SLE registry were analyzed using hospital medical records. Organ manifestations, laboratory parameters, and immunoserological characteristics were reviewed and the results were evaluated using SPSS for Windows software.Gender distribution was not significantly different between groups with disease starting in childhood vs adulthood. The prevalence of the following manifestations was significantly higher for pediatric than for adult-onset disease including: lupus nephritis (43% pediatric vs 26.4% for adult-onset), hematological disorders (57% vs 36.4%), photosensitivity (20% vs 9%), butterfly rash (61% vs 35.5%) and mucosal ulceration (11.4% vs 4%). For adult-onset SLE, neurological symptoms (30% vs 6%) and polyarthritis (86% vs 68%) occurred significantly more frequently than in children. Anti-SSA, anti-SSB and antiphospholipid antibodies were detected at significantly higher levels in adult-onset patients compared to those in pediatrics. Children were more commonly given high-dose intravenous immunoglobulin treatment (6.3% vs 0.6%) and mycophenolate mofetil (15.2% vs 5.3%) than adults.These results suggest that pediatric and adult-onset SLE differ in multiple aspects, and it is important to recognize these differences for optimal treatment and prognosis of these patients.

摘要

系统性红斑狼疮(SLE)是一种多因素自身免疫性疾病,在育龄女性中患病率最高。然而,16岁以下的儿童也可能患上SLE(儿童期发病的狼疮/青少年型SLE)。我们研究的目的是比较成人和儿童期发病的SLE的临床病程。使用医院病历分析了匈牙利德布勒森大学随访的342例成年患者以及匈牙利国家儿童SLE登记处记录的79例儿童的数据。回顾了器官表现、实验室参数和免疫血清学特征,并使用SPSS for Windows软件评估结果。儿童期发病与成年期发病的两组患者性别分布无显著差异。以下表现的患病率在儿童期发病的疾病中显著高于成年期发病的疾病,包括:狼疮性肾炎(儿童期发病为43%,成年期发病为26.4%)、血液系统疾病(57%对36.4%)、光敏性(20%对9%)、蝶形红斑(61%对35.5%)和黏膜溃疡(11.4%对4%)。对于成年期发病的SLE,神经症状(30%对6%)和多关节炎(86%对68%)的发生频率明显高于儿童。与儿童相比,成年期发病患者中抗SSA、抗SSB和抗磷脂抗体的检测水平显著更高。与成人相比,儿童更常接受大剂量静脉注射免疫球蛋白治疗(6.3%对0.6%)和霉酚酸酯治疗(15.2%对5.3%)。这些结果表明,儿童期和成年期发病的SLE在多个方面存在差异,认识到这些差异对于这些患者的最佳治疗和预后非常重要。

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