单中心171例系统性红斑狼疮住院患儿起病特征分析  

A single-center study of clinical characteristics of 171 hospitalized children with systemic lupus erythematosus

作　　者：朱国豪 王大海 林毅[1] 张冉冉 王影 常红[1] ZHU Guohao;WANG Dahai;LIN Yi;ZHANG Ranran;WANG Ying;CHANG Hong(Department of Nephrology,Rheumatology and Immunology,The Affiliated Hospital of Qingdao University,Qingdao 266003,China)

机构地区：[1]青岛大学附属医院儿童肾脏风湿免疫科,山东青岛266003

出　　处：《精准医学杂志》2024年第3期275-278,282,共5页Journal of Precision Medicine

基　　金：“十四五”国家重点研发计划课题(2021YFC270-2004)。

摘　　要：目的探讨我院10年间儿童系统性红斑狼疮(childhood-onset systemic lupus erythematosus,cSLE)住院患儿的临床起病特点及其与性别、抗可溶性抗原(ENA)抗体谱的相关性。方法选择2012年1月—2021年12月我院住院的cSLE患儿171例,收集其年龄、性别、首发症状、系统受累情况及血清中补体C3、补体C4、免疫球蛋白、抗ENA抗体谱等资料,分析患儿起病特征,比较上述指标的男女差异及抗ENA抗体谱与受累器官的相关性。结果患儿确诊前误诊率为43.27%,首发症状以皮疹和发热为主(各占28.31%)。73.68%的患儿血液系统受累,女性发生贫血比例明显高于男性(χ^(2)=3.863,P<0.05)。61.54%的患儿肾脏受累,男性血尿合并蛋白尿比例明显高于女性(χ^(2)=10.373,P<0.05),女性发生孤立性蛋白尿比例明显高于男性(χ^(2)=5.718,P<0.05)。抗ENA抗体谱中,抗ds-DNA抗体、抗组蛋白抗体、抗核小体抗体阳性患儿出现肾脏受累的比例更高(χ^(2)=5.306~12.667,P<0.05);抗Sm抗体、抗核糖核酸蛋白抗体阳性患儿出现皮肤黏膜系统受累的比例更高(χ^(2)=4.495、4.210,P<0.05),出现血液系统受累的比例则更低(χ^(2)=5.618、6.719,P<0.05)。结论cSLE误诊率高,首发症状以发热和皮疹为主,易累及肾脏和血液系统,且男女间的系统受累情况存在差异。cSLE患儿抗ENA抗体谱中部分抗体阳性与肾脏、皮肤黏膜、血液系统受累相关。Objective To investigate the clinical characteristics at childhood-onset systemic lupus erythematosus(cSLE)in our hospital over the past decade,and to analyze the relationship with sex and anti-extractable nuclear antigen(ENA)antibodies.[WTHX]Methods We included 171 children with cSLE admitted to our hospital from January 2012 to December 2021,collecting their data on age,sex,onset symptoms,and system involvement,as well as serum complements C3 and C4,immunoglobulin,and ENA antibodies.We analyzed the clinical features at disease onset,compared the above indicators between sexes,and determined the relationship between anti-ENA antibodies and organ/system involvement.Results The misdiagnosis rate before the definitive diagnosis of the children was 43.27%.The initial symptoms were mainly rashes and fever(28.31%each).Hematological involvement occurred in 73.68%of the children.The proportion of anemia was significantly higher in females than in males(χ^(2)=3.863,P<0.05).Renal involvement occurred in 61.54%of the children.The proportion of hematuria with proteinuria in males was significantly higher than that in females(χ^(2)=10.373,P<0.05).Isolated proteinuria was significantly more frequent in females than in males(χ^(2)=5.718,P<0.05).Across the anti-ENA antibody spectrum,the presence of anti-double stranded DNA,anti-histone,or anti-nucleosome antibodies was significantly associated with a higher proportion of kidney involvement(χ^(2)=5.306-12.667,P<0.05);and the presence of anti-Smith or anti-ribonucleoprotein antibodies was significantly associated with a higher proportion of skin and mucosal system involvement(χ^(2)=4.495,4.210,P<0.05)and a lower proportion of hematological involvement(χ^(2)=5.618,6.719,P<0.05).Conclusion cSLE is easily misdiagnosed,which typically begins with fever and rashes and frequently affects the kidney and hematological system,with sex-related differences in system involvement.Certain anti-ENA antibodies are associated with renal,skin and mucosa,and hematological involvement in cSLE.

关 键 词：红斑狼疮 系统性 儿童 前驱症状 抗原 核 性别因素 

分 类 号：R725.9[医药卫生—儿科] R593.241[医药卫生—临床医学]