儿童期起病的男性系统性红斑狼疮患儿30例临床特征分析  

Clinical characteristics of thirty male childhood-onset systemic lupus erythematosus

作　　者：张宇[1] 唐雪梅[1] 汪利 王雅婷[1] 徐莉[1] 罗冲[1] ZHANG Yu;TANG Xue-mei;WANG Li(Department of Rheumatology and Immunology,Children’s Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Development and Disorders,National Clinical Research Center for Child Health and Disorders,China International Science and Technology Cooperation Base of Child Development and Critical Disorders,Chongqing Key Laboratory of Child Infection and Immunity,Chongqing400014,China)

机构地区：[1]重庆医科大学附属儿童医院风湿免疫科儿童发育疾病研究教育部重点实验室国家儿童健康与临床医学研究中心儿童发育重大疾病国家国际科技合作基地儿童感染免疫重庆市重点实验室,重庆400014

出　　处：《中国实用儿科杂志》2020年第7期547-551,共5页Chinese Journal of Practical Pediatrics

摘　　要：目的探讨儿童期起病男性系统性红斑狼疮(SLE)患儿临床特点及预后。方法回顾性分析2011年1月至2016年10月于重庆医科大学附属儿童医院住院初诊为SLE的男性患儿临床资料及预后,与同期初诊女性SLE患儿进行比较。结果 SLE患儿男30例,女129例,中位随访时间45个月。男性患儿平均年龄(11.57±2.78)岁,肝脾大为最常见首发表现(70.0%),其次为皮肤黏膜表现、发热、淋巴结肿大、血液系统受累、肾脏受累,发生率均高于50%。其中肝脾大、浆膜炎及口腔溃疡者发生率均高于女性(P<0.05)。13例男性SLE患儿完成肾活检,Ⅳ型狼疮性肾炎发生率为76.9%,与女性患儿差异无统计学意义(P>0.05)。男性患儿出现慢性肾脏病Ⅲ期及以上者高于女性患儿(P<0.05),2例死亡病例死因为肾功能衰竭。不同性别患儿存活率及并发症发生率差异无统计学意义(P>0.05)。结论男性SLE患儿首发表现肝脾大、浆膜炎及口腔溃疡较女性患儿多见。男性SLE患儿远期更易发生严重的肾脏损害,且为男性SLE患儿死亡的主要原因。Objective To investigate the clinical characteristics and prognosis of male childhood-onset systemic lupus erythematosus(cSLE).Methods Clinical data and prognosis of male childhoond-onset SLE(cSLE)who were hospitalized in our hospital from January 2011 to October 2016 were retrospectively analyzed and compared with those of female cSLE diagnosed in our hospital.Results There were 30 cases of male cSLE and 129 cases of female patients.The median follow-up time was 45 months.The average age of male cSLE were(11.57±2.78)years.Hepatosplenomegaly was the most common initial manifestation(70.0%),followed by mucocutaneous involvement,fever,lymphadenopathy,haemocytopenia and renal involvement,the incidence being higher than 50%.The incidences of hepatosplenomegaly,serositis and oral ulcer in male cSLE were higher than those in female cSLE(P<0.05).Thirteen male cSLE completed renal biopsy,and 76.9%of them had Class IV lupus nephritis,and there was no significant difference in renal histopathology between male and female cSLE(P>0.05).The incidence of stageⅢor above chronic kidney disease in male cSLE was higher than that in female cSLE(P<0.05).Two cases of male cSLE died and the death cause was renal failure.There were no significant differences in survival rates incidence of complications between male cSLE and female cSLE(P>0.05).Conclusion Hepatosplenomegaly,serositis and oral ulcer are more common in male cSLE than in female cSLE.Male cSLE patients are more likely to suffer from severe kidney damage in the long term,which is the main cause of death in male cSLE.

关 键 词：儿童 男性 系统性红斑狼疮 临床特征 预后 

分 类 号：R72[医药卫生—儿科]