本文报告14例小儿狼疮性肾炎(LN),临床表现:11例为肾病综合征,2例为肾炎综合征,1例为单纯性血尿。临床与病理诊断的关系:11例肾病综合征中7例为Ⅳ型,Ⅱ型、Ⅲ型各2例;2例肾炎综合征均为Ⅳ型;1例单纯性血尿为Ⅱ型。14例中Ⅳ型共9例(64.3％)。4例CANCA阳性(>1:20)。6例肾病综合征(Ⅳ)采用CTX冲击治疗,3例缓解,1例部分缓解,2例继续治疗中。 This article reports 14 cases of children with lupus nephritis (LN), clinical manifestations: 11 cases of nephrotic syndrome, 2 cases of nephritic syndrome, 1 case of simple hematuria. Clinical and pathological diagnosis of the relationship between: 11 cases of nephrotic syndrome in 7 cases of type Ⅳ, type Ⅱ and type Ⅲ in 2 cases; 2 cases of nephritic syndrome are type Ⅳ; 1 case of simple hematuria type Ⅱ. In 14 cases, type Ⅳ was found in 9 cases (64.3%). 4 cases of CANCA positive (> 1:20). Six patients with nephrotic syndrome (Ⅳ) were treated with CTX shock, three were relieved, one was partially relieved and two were continued treatment.