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目的探讨儿童SLE的临床特点,分析SLE疾病活动性指数(SLEDAI)及疗效,为儿童SLE的诊疗提供临床依据。方法回顾性分析1999年1月-2010年2月住院的111例SLE患儿资料,总结其临床表现,实验室检查、病情活动性评分及治疗,并对40例正规随访病例的SLEDAI进行随访,了解治疗效果。结果儿童SLE临床表现以皮疹(66%)、发热(59%)及关节炎(32%)最常见。器官损害以肾脏损害(59%)及血液系统损害(52%)多见。肾活检以WHOⅣ型最多见,病理改变主要为弥散增殖型和系膜增殖型,多伴有肾小管间质病变。免疫学检查发现免疫球蛋白增高以IgG增高最为显著(59%),自身抗体阳性率高,以抗核抗体(ANA)(69%)、抗ds-DNA(63%)、抗ss-DNA(75%)、抗Sm(52%)阳性最常见。儿童SLE治疗仍以糖皮质激素为主,可根据病情轻重不同,选择甲泼尼龙和(或)环磷酰胺冲击治疗,或加用其他免疫抑制剂,疗程较长。SLEDAI评分提示儿童SLE发病多为中-重度活动,且起病形式多样。对随访病例进行SLEDAI评分,发现平均分有所下降,提示治疗有效。结论儿童SLE的临床表现复杂,诊断较困难,应完善相关检查,尽早确诊、治疗,改善预后。SLEDAI在儿科应用的准确性和实用性有待考证。
Objective To explore the clinical features of children with SLE, analysis of SLE disease activity index (SLEDAI) and efficacy, provide clinical evidence for the diagnosis and treatment of children with SLE. Methods The data of 111 SLE children admitted to hospital from January 1999 to February 2010 were retrospectively analyzed. The clinical manifestations, laboratory tests, assessment of disease activity and treatment were reviewed. The follow-up of SLEDAI in 40 patients with normal follow- Understand the treatment effect. Results The clinical manifestations of children with rashes (66%), fever (59%) and arthritis (32%) were the most common. Organ damage is more common in kidney damage (59%) and blood system damage (52%). Renal biopsy is the most common WHO Ⅳ type, pathological changes mainly diffuse proliferative and mesangial proliferative, and more associated with tubulointerstitial lesions. The results of immunological examination showed that the increase of immunoglobulin was the most significant (59%) in IgG, the autoantibody positive rate was high. Anti-nuclear antibody (ANA) 69%, anti-ds DNA 63% 75%), anti-Sm (52%) positive the most common. Children with SLE treatment is still glucocorticoid-based, depending on the severity of the disease, choose methylprednisolone and (or) cyclophosphamide impact treatment, or add other immunosuppressive agents, longer course of treatment. SLEDAI score prompted children with SLE onset mostly in the - severe activity, and the onset of various forms. SLEDAI score of follow-up cases found that the average score decreased, suggesting that the treatment is effective. Conclusion The clinical manifestations of children with SLE complicated diagnosis is more difficult, should improve the relevant examination, early diagnosis and treatment, improve prognosis. The accuracy and usefulness of SLEDAI in pediatric applications remains to be verified.