临床儿科杂志 ›› 2023, Vol. 41 ›› Issue (12): 908-913.doi: 10.12372/jcp.2023.23e0276

• 泌尿系统疾病专栏 • 上一篇    下一篇

儿童狼疮性肾炎临床及病理特点与预后分析

殷秋霞, 成学琴(), 赵非, 丁桂霞, 赵三龙, 朱春华   

  1. 南京医科大学附属儿童医院肾脏科(江苏南京 210008)
  • 收稿日期:2023-04-04 出版日期:2023-12-15 发布日期:2023-12-04
  • 通讯作者: 成学琴 电子信箱:duoer417@126.com

Clinical and pathological characteristics and prognosis of lupus nephritis in children

YIN Qiuxia, CHENG Xueqin(), ZHAO Fei, DING Guixia, ZHAO Sanlong, ZHU Chunhua   

  1. Department of Nephrology, Children's Hospital of Nanjing Medical University, Nanjing 210008, Jiangsu, China
  • Received:2023-04-04 Online:2023-12-15 Published:2023-12-04

摘要:

目的 分析狼疮性肾炎(LN)患儿的临床特征及影响疗效和预后的因素。方法 回顾性分析2010年7月1日至2019年12月31日在肾脏科初诊的LN患儿的临床资料。结果 LN患儿98例,男19例、女79例,平均发病年龄(11.0±2.0)岁。肾脏表现中蛋白尿91例,血尿84例,不同程度水肿37例,伴高血压21例。肾外表现中,皮疹73例,伴狼疮性脑病9例;白细胞下降、贫血及血小板降低分别有82、71和87例。与非肾病综合征(NS)型组相比,NS组男性、发热、血尿、合并急性肾损伤、抗双链DNA抗体阳性、血小板减少比例较高,差异有统计学意义(P<0.05)。98例患儿中,初始诱导方案为泼尼松+环磷酰胺61例,泼尼松+吗替麦考酚酯22例,泼尼松+他克莫司8例,泼尼松+硫唑嘌呤3例,单纯泼尼松4例。行肾活检的96例患儿中病理分型为Ⅱ型4例、Ⅲ型9例、Ⅳ型45例、Ⅴ型8例、Ⅳ+Ⅴ型25例、Ⅲ+Ⅴ型5例。从疾病初发至治疗6个月时肾脏完全缓解81例(82.7%),部分缓解12例,未缓解5例。与完全缓解组相比,未完全缓解组依从性不规则比例较高,差异有统计学意义(P<0.05)。92例患儿完成随访,中位随访时间5.2(1.8~5.2)年,复发64例(69.6%)。与未复发组相比,复发组依从性不规则、>9岁、合并高血压、合并急性肾损伤、抗双链DNA抗体阳性的比例较高,差异有统计学意义(P<0.05)。结论 LN患儿临床表现多样,疗效与患儿依从性有关;复发与患儿年龄、依从性、合并高血压和急性肾损伤以及抗双链DNA抗体阳性有关。

关键词: 狼疮性肾炎, 缓解, 预后, 儿童

Abstract:

Objective To analyze the clinical features of children with lupus nephritis (LN) and the factors affecting curative effect and prognosis. Methods The clinical data of children with LN who were newly diagnosed in the nephrology department from July 1, 2010 to December 31, 2019 were retrospectively analyzed. Results Ninety-eight children (19 boys and 79 girls) with LN were included, and the mean age of onset was (11.0±2.0) years. Of the 98 children with LN, 91 were accompanied by proteinuria, 84 were accompanied by hematuria, 37 were accompanied by varying degrees of edema and 21 were accompanied by hypertension. Among the extrarenal manifestations, 73 children had rash and 9 had lupus encephalopathy. There were 82 cases of leukopenia, 71 cases of anemia and 87 cases of thrombocytopenia. Compared with non-nephrotic syndrome (NS) group, the proportions of male, fever, hematuria, acute kidney injury, positive anti-double-stranded DNA antibody, and thrombocytopenia in NS group were higher, and the differences were statistically significant (P<0.05). Among the 98 children, the initial induction scheme was prednisone + cyclophosphamide in 61 cases, prednisone + mycophenolate mofetil in 22 cases, prednisone + tacrolimus in 8 cases, prednisone + azathioprine in 3 cases, and prednisone alone in 4 cases. Among the 96 children who underwent renal biopsy, the pathological classification was type Ⅱ in 4 cases, type Ⅲ in 9 cases, type Ⅳ in 45 cases, type Ⅴ in 8 cases, type Ⅳ + type Ⅴ in 25 cases, and type Ⅲ + type Ⅴ in 5 cases. From the onset of the disease to 6 months of treatment, 81 children (82.7%) had complete remission, 12 had partial remission, and 5 had no remission. Compared with the complete remission group, the proportion of irregular compliance in the non-complete remission group was higher, and the difference was statistically significant (P<0.05). A total of 92 patients were followed up, the median follow-up time was 5.2 (1.8-5.2) years, and 64 patients (69.6%) recurred. Compared with the non-recurrence group, the proportion of children with irregular compliance, >9 years old, complicated hypertension, complicated acute kidney injury and positive anti-double-stranded DNA antibody in the recurrence group was higher, and the difference was statistically significant (P<0.05). Conclusions The clinical manifestations of LN children are varied, and the curative effect is related to the compliance of the children. Recurrence was associated with age, compliance, combination of hypertension and acute kidney injury, and positive anti-double-stranded DNA antibody.

Key words: lupus nephritis, remission, prognosis, child