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目的 回顾分析狼疮肾炎患儿的临床资料及长期生存状况 ,探讨儿童狼疮肾炎 (LN)的临床特点、分析影响其预后和长期生存率的相关因素。方法 收集自 1990～ 2 0 0 1年住院治疗、符合美国风湿病学会 (1982年 )关于系统性红斑狼疮 (SLE)及其肾损害诊断标准、年龄小于 18岁的患儿资料 ,对其进行回顾性分析。结果 92例SLE儿童中 ,合并LN者 5 6例 ,发生率为 6 0 9% ;年龄 4 8～ 15 9岁 ,平均年龄 (12 8± 2 3)岁 ;男女比例 3∶1。5 6例LN中临床以水肿或尿异常起病者 11例 (19 6 % ) ,以发热、皮疹等全身症状起病者 4 5例 (80 4 % ) ;发生LN的高危因素为血浆抗双链DNA(dsDNA)抗体升高和低补体。有完整随访资料的 4 8例 ,平均随访 (4 92± 2 6 3)年 ,其 1年、5年和 >10年的肾脏生存率分别为 94 8%、89 5 %、81 9% ;男性、贫血、SLE疾病活动指数≥ 2 0、肾脏病理评分活动性指数≥ 7、慢性指数≥ 4和病理分型Ⅳ型是影响LN预后的重要因素。结论 在儿童SLE中 ,LN发生率高 ;其发生的高危因素是血浆dsDNA抗体升高和补体降低。积极合理个体化的治疗可明显改善预后。感染仍然是儿童LN死亡的主要原因。
Objective To retrospectively analyze the clinical data and long-term survival of children with lupus nephritis and to explore the clinical features of children with lupus nephritis (LN) and to analyze the related factors that affect their prognosis and long-term survival rate. Methods The data collected from 1990 to 2001 in hospital and in line with the American College of Rheumatology (1982) criteria for the diagnosis of systemic lupus erythematosus (SLE) and its renal impairment, were analyzed retrospectively Sexual analysis. Results Of the 92 children with SLE, 56 cases were complicated with LN, the incidence rate was 60.9%. The mean age was from 48 to 15 years (mean age = 12 8 ± 23). The male to female ratio was 3 to 1.5. Eleven patients (19.6%) were clinically diagnosed as having edema or abnormal urine in LN, and 45 (80.4%) were diagnosed as systemic symptoms such as fever and rash. The high risk factors of LN were plasma anti-double stranded DNA dsDNA) antibody and low complement. Forty-eight patients with complete follow-up data, with an average follow-up of (4 92 ± 2 6 3) years, had renal survival rates of 94.8%, 89.5% and 81.9% at 1 year, 5 years and> , Anemia, SLE disease activity index ≥ 20, renal pathological score activity index ≥ 7, chronic index ≥ 4 and pathological type Ⅳ are the important factors affecting LN prognosis. Conclusion The incidence of LN is high in children with SLE. The high risk factors of the disease are the increase of plasma dsDNA antibody and the decrease of complement. Positive and rational individualized treatment can significantly improve the prognosis. Infection is still the leading cause of LN death in children.