儿童狼疮性肾炎的病理与临床研究

摘要

Toimprovetheclinicalunderstandingandtreatmentoflupusnephritis(LN)inchildren,toinvesti-gate the relationship between pathological classification and clinical manifestation of LN,and therapeutic responses. Methods:To retrospectively analyze patients with LN before18years of age,during 1990 -2000 year in hospital. The renal biopsy was made in 47 patients with LN. Pathological classification was based upon the stan-dard criteria of World Health Organism (WHO)1982,and scored their active index(AI),chronic index(CI). To investigate clinical manifestation and pathological features and therapeutic results. Results:Type Ⅳ was nost common(53. 2%),typeⅡ(19. 1%)amd tupeⅤ(19. 1%)were next. Type Ⅱ usually manifested as hematuria and/or proteinuria,on the other hand,severe proteinuria was found in Ⅳ,Ⅲtype LN. InⅣ type LN,there was high incidence of hypertension and extrarenal anifestations,and a highest AI. Ninety-three percent of the patients were treated with corticosteroids and pulse cyclophosphamide. The mean follow-up was(41. 6 ± 29. 7) months,the rate of remission was 88. 4%. The remission was higher and the time to remission was short inⅡ,Ⅳ type than inⅢ,Ⅴ type LN. Conclusion:A positive correlation is demonstrated between pathological classification and clinical manifesta-tions of LN,pathological classification could be foretold according to the clinical manifestation and necessary laboratory parame-ters. Renal biopsy is important in diagnosis of active status,in designing of therapeutic regimen and predicting of prognosis in LN.%目的：总结儿童不同病理改变的狼疮性肾炎（ LN）的临床特点以及治疗效果，以提高临床诊治水平。方法：总结12年来收治的年龄﹤18岁狼疮性肾炎患者的临床资料（且有肾活组织检查资料者），根据世界卫生组织（ WHO）1982年标准进行病理分型，并分别进行活动性指数（ AI）和慢性指数（ CI）评分。结果：47例LN的病理类型包括Ⅱ型9例（19.1%），Ⅲ型4例（8.5%），Ⅳ型25例（53.2%）和Ⅴ型9例（19.1%）。其中Ⅳ型AI显著高于其他病理类型。Ⅱ型LN主要表现为血尿和（或）蛋白尿，预后好；Ⅲ型、Ⅳ型多表现为大量蛋白尿，其中Ⅳ型伴高血压及多系统受累的比例高。43例LN平均随访（41.6±29.7）个月，其中应用激素加环磷酰胺冲击（ CTX-IV）治疗40例，1年内缓解率76.7%。Ⅱ型、Ⅳ型多在1年内缓解，Ⅲ型和Ⅴ型短期缓解率低。结论：LN的病理类型与临床表现有一定关系，根据其临床表现和实验室检查结果可以大致推测其病理类型。而肾活组织检查对于准确判断疾病活动性、指导治疗与估计预后有重要意义。