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目的探讨儿童系统性红斑狼疮(SLE)的临床特点。方法对26例儿童SLE患者的临床资料进行回顾性研究,分析其临床表现、辅助检查结果等临床特征及误诊率。结果男女比例1∶4.2,平均年龄12.6岁。26例患者首发症状中发热、乏力和体质量减轻等非特异性症状占42.3%,皮肤黏膜表现占26.9%,关节肌肉症状占26.9%。脏器受累中肾脏损害占69.2%,皮肤、黏膜损害占38.5%,骨骼肌肉受累占34.6%,血液系统损害占26.9%。10例在疾病早期曾有误诊,误诊率达38.5%。ANA、dsDNA阳性率分别为88.5%与69.2%,C3降低为61.5%,血沉增快为76.9%。结论儿童SLE典型初发症状主要为不明原因的发热、皮疹、关节痛和体质量减轻。器官受累以肾脏最为常见,其次为皮肤、黏膜,再次为骨骼肌肉系统及血液系统。该病临床表现多样且多不典型,易误诊。多系统受累表现、免疫指标检查以及血常规、血沉、尿液检查等异常有助于提示诊断。
Objective To investigate the clinical features of children with systemic lupus erythematosus (SLE). Methods The clinical data of 26 children with SLE were retrospectively analyzed, and the clinical features, misdiagnosis rates and other clinical features were analyzed. Results The male-female ratio was 1: 4.2 with an average age of 12.6 years. Twenty-six patients had non-specific symptoms such as fever, fatigue and weight loss in the first symptom, accounting for 42.3%, skin and mucous membranes accounting for 26.9%, and joint and muscle symptoms accounting for 26.9%. Organ involvement kidney damage accounted for 69.2%, skin, mucosal damage accounted for 38.5%, skeletal muscle involvement accounted for 34.6%, blood system damage accounted for 26.9%. 10 cases had been misdiagnosed early in the disease, misdiagnosis rate reached 38.5%. The positive rates of ANA and dsDNA were 88.5% and 69.2%, C3 was 61.5%, and erythrocyte sedimentation rate was 76.9%. Conclusion The typical primary symptoms of SLE in children are mainly fever, rash, joint pain and weight loss with unknown causes. Organ involvement is most common in the kidneys, followed by the skin, mucous membranes, and again the skeletal muscle system and the blood system. The clinical manifestations of the disease and more atypical, easily misdiagnosed. Multiple systems involved in performance, immune indicators and blood tests, ESR, urine tests and other anomalies help prompt diagnosis.