儿童系统性红斑狼疮的临床特点

CLINICAL FEATURES OF SYSTEMIC LUPUS ERYTHEMATOSUS IN CHILDREN

目的探讨儿童系统性红斑狼疮(SLE)的临床特点,提高诊疗水平。方法回顾性分析32例SLE病儿的临床资料。结果 32例SLE病儿,女∶男=5.4∶1,中位数年龄为11.5岁;首发症状以皮疹(37.5%)、发热(37.5%)、关节痛(15.6%)、水肿(12.5%)最多见;临床表现以发热(71.9%)、皮疹(62.5%)、水肿(28.1%)、头痛(28.1%)、出血(25.0%)、关节痛(25.0%)最多见。辅助检查:血液系统以贫血最多见(40.6%);泌尿系统以血尿(56.3%)、蛋白尿(43.8%)最多见;免疫学指标以抗核抗体(ANA)阳性(96.9%)、抗双链DNA抗体(dsDNA)阳性(84.4%)、抗核小体抗体阳性(43.8%)、抗SSA抗体阳性(34.4%)多见;C3下降(65.6%),C4下降(59.4%),ESR增快(62.5%)。易误诊疾病依次为特发性血小板减少性紫癜、不明原因发热、过敏性紫癜、肾病综合征等。结论儿童SLE临床表现复杂,以发热、皮疹、泌尿系统和神经系统损害最多见,其中狼疮性肾炎是儿童SLE诊治重点。

Objective To investigate clinical features of systemic lupus erythematosus（SLE） in children and raise the level of diagnosis and treatment of this disease. Methods A retrospective analysis was done for clinical data in 32 children with SLE. Results Of the 32 sick kids,male female ratio was 1∶5.4,and a median age was 11.5 years.The first manifestations that most commonly seen were skin rash（37.5%）,fever（37.5%）,arthralgia（15.6%）,and edema（12.5%）.Clinical manifestations were fever（71.9%）,skin rash（62.5%）,edema（28.1%）,headache（28.1%）,hemorrhage（25.0%）,and arthralgia（25.0%）.Auxiliary examinations： hematologically,anemia（40.6%） was the mostly seen;for urinary system,hematuria（56.3%） and proteinuria（43.8%） were the most.Immunologically： positive expressions were found in antinuclear antibody（96.9%）,anti-double stranded DNA antibody（84.4%）,anti-nucleosome antibody（43.8%）,and anti-SSA antibody（34.4%）.Both C3 and C4 decreased（65.6% and 59.4%,respectively）,ESR increased（62.5%）.The diseases that were easily to be misdiagnosed were in order of idiopathic thrombocytopenic purpura,fever of undetermined origin,allergic purpura,and nephritic syndrome. Conclusion The clinical manifestations of SLE in children are complicated.Fever,skin rash,impairment of urinary and nervous system are the most common ones,in which,the focus point should be put on the diagnosis and therapy of lupus nephritis.