泌尿系统疾病专栏

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
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  • 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

  网络出版日期: 2023-12-04

Clinical and pathological characteristics and prognosis of lupus nephritis in children

  • Qiuxia YIN ,
  • Xueqin CHENG ,
  • Fei ZHAO ,
  • Guixia DING ,
  • Sanlong ZHAO ,
  • Chunhua ZHU
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  • Department of Nephrology, Children's Hospital of Nanjing Medical University, Nanjing 210008, Jiangsu, China

Received date: 2023-04-04

  Online 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。

本文引用格式

殷秋霞 , 成学琴 , 赵非 , 丁桂霞 , 赵三龙 , 朱春华 . 儿童狼疮性肾炎临床及病理特点与预后分析[J]. 临床儿科杂志, 2023 , 41(12) : 908 -913 . DOI: 10.12372/jcp.2023.23e0276

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.

参考文献

[1] Mejia-Vilet JM, Malvar A, Arazi A, et al. The lupus nephritis management renaissance[J]. Kidney Int, 2022, 101(2): 242-255.
[2] 张爱华, 朱春华. 儿童狼疮性肾炎诊治现状及面临的困难[J]. 中华儿科杂志, 2021, 59(9): 717-719.
[3] Montigny PM, Houssiau FA. New treatment options in lupus nephritis[J]. Arch Immunol Ther Exp (Warsz), 2022, 70(1): 11.
[4] 中华医学会儿科学分会肾脏学组. 狼疮性肾炎诊治循证指南(2016)[J]. 中华儿科杂志, 2018, 56(2): 88-94.
[5] Wu LH, Yu F, Tan Y, et al. Inclusion of renal vascular lesions in the 2003 ISN/RPS system for classifying lupus nephritis improves renal outcome predictions[J]. Kidney Int, 2013, 83(4): 715-723.
[6] Radhakrishnan J, Cattran DC. The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient[J]. Kidney Int, 2012, 82(8): 840-856.
[7] 孙莉, 徐虹, 刘海梅, 等. 上海单中心101例儿童狼疮性肾炎的长期随访分析[J]. 中华儿科杂志, 2011, 49(11): 820-824.
[8] Gallan AJ, Chang A. A new paradigm for renal thrombotic microangiopathy[J]. Semin Diagn Pathol, 2020, 37(3): 121-126.
[9] Gomes RC, Silva MF, Kozu K, et al. Features of 847 childhood-onset systemic lupus erythematosus patients in three age groups at diagnosis: a Brazilian multicenter study[J]. Arthritis Care Res (Hoboken), 2016, 68(11): 1736-1741.
[10] Couture J, Silverman ED. Update on the pathogenesis and treatment of childhood-onset systemic lupus erythematosus[J]. Curr Opin Rheumatol, 2016, 28(5): 488-496.
[11] Ameer MA, Chaudhry H, Mushtaq J, et al. An overview of systemic lupus erythematosus (SLE) pathogenesis, classification, and management[J]. Cureus, 2022, 14(10): e30330.
[12] Parikh SV, Almaani S, Brodsky S, et al. Update on lupus nephritis: core curriculum 2020[J]. Am J Kidney Dis, 2020, 76(2): 265-281.
[13] Strufaldi FL, Menezes Neves PDMM, Dias CB, et al. Renal thrombotic microangiopathy associated to worse renal prognosis in lupus nephritis[J]. J Nephrol, 2021, 34(4): 1147-1156.
[14] Pinheiro SVB, Dias RF, Fabiano RCG, et al. Pediatric lupus nephritis[J]. J Bras Nefrol, 2019, 41(2): 252-265.
[15] Onuora S. LLDAS is an attainable SLE treatment target[J]. Nat Rev Rheumatol, 2019, 15(11): 636.
[16] van Vollenhoven R, Voskuyl A, Bertsias G, et al. A framework for remission in SLE: consensus findings from a large international task force on definitions of remission in SLE (DORIS)[J]. Ann Rheum Dis, 2017, 76(3): 554-561.
[17] Massicotte-Azarniouch D, Kotzen E, Todd S, et al. Kidney thrombotic microangiopathy in lupus nephritis: impact on treatment and prognosis[J]. Lupus, 2022, 31(10): 1175-1185.
[18] Almaani S, Parikh SV. Membranous lupus nephritis: a clinical review[J]. Adv Chronic Kidney Dis, 2019, 26(5): 393-403.
[19] Chen X, Cheng W, Wang G, et al. Clinical characteristics and prognosis of renal thrombotic microangiopathy in lupus nephritis[J]. Iran J Kidney Dis, 2021, 15(3): 169-176.
[20] Massicotte-Azarniouch D, Kotzen E, Todd S, et al. Kidney thrombotic microangiopathy in lupus nephritis: impact on treatment and prognosis[J]. Lupus, 2022, 31(10): 1175-1185.
[21] Oni L, Wright RD, Marks S, et al. Kidney outcomes for children with lupus nephritis[J]. Pediatr Nephrol, 2021, 36(6): 1377-1385.
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