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中华妇幼临床医学杂志(电子版) ›› 2026, Vol. 22 ›› Issue (03) : 214 -221. doi: 10.3877/cma.j.issn.1673-5250.2026.03.004

论著

血清抗中性粒细胞细胞质抗体呈阳性狼疮性肾炎患儿的临床分析
李华荣, 陈朝英(), 杨宏仙, 涂娟, 耿海云, 万灵, 孙金山   
  1. 首都医科大学附属首都儿童医学中心肾脏内科,北京 100020
  • 收稿日期:2025-03-10 修回日期:2026-04-22 出版日期:2026-06-01
  • 通信作者: 陈朝英

Clinical analysis of pediatric lupus nephritis patients with positive serum antineutrophil cytoplasmic antibody

Huarong Li, Chaoying Chen(), Hongxian Yang, Juan Tu, Haiyun Geng, Ling Wan, Jinshan Sun   

  1. Department of Nephrology, Capital Center for Children′s Health, Capital Medical University, Beijing 100020, China
  • Received:2025-03-10 Revised:2026-04-22 Published:2026-06-01
  • Corresponding author: Chaoying Chen
引用本文:

李华荣, 陈朝英, 杨宏仙, 涂娟, 耿海云, 万灵, 孙金山. 血清抗中性粒细胞细胞质抗体呈阳性狼疮性肾炎患儿的临床分析[J/OL]. 中华妇幼临床医学杂志(电子版), 2026, 22(03): 214-221.

Huarong Li, Chaoying Chen, Hongxian Yang, Juan Tu, Haiyun Geng, Ling Wan, Jinshan Sun. Clinical analysis of pediatric lupus nephritis patients with positive serum antineutrophil cytoplasmic antibody[J/OL]. Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition), 2026, 22(03): 214-221.

目的

探讨血清抗中性粒细胞细胞质抗体(ANCA)呈阳性狼疮性肾炎(LN)患儿的临床表现、肾组织病理学特征及预后。

方法

选择2015年1月至2024年2月在首都医科大学附属首都儿童医学中心肾脏内科住院并经肾组织活检确诊的53例新发LN患儿为研究对象。根据其血清ANCA结果,将其分别纳入研究组(n=9,血清ANCA结果呈阳性)和对照组(n=44,血清ANCA结果呈阴性)。采用回顾性分析方法,收集2组患儿临床表现、实验室检查结果、肾组织病理学特征及预后等临床资料,并采用成组t检验、Mann-Whitney U检验、χ2检验、Fisher确切概率法等进行统计学比较。本研究经首都医科大学附属首都儿童医学中心伦理委员会批准(审批文号:SHERLL2021052)。

结果

①本研究53例LN患儿中,男、女性患儿分别为19、34例,发病年龄为(11.2±2.8)岁。研究组与对照组患儿的性别构成比、发病年龄等一般临床资料分别比较,差异均无统计学意义(P>0.05)。②与对照组相比,研究组患儿的血红蛋白(Hb)水平更低[79.0 g/L(68.0,88.5 g/L) vs 102.0 g/L(84.3,113.8 g/L)],而系统性红斑狼疮疾病活动指数2000(SLEDAI-2000)评分[23.0分(18.0,30.0分)vs 18.0分(16.0,20.8分)]与红细胞沉降率(ESR)[(86.1±43.5) mm/h vs(38.7±27.7) mm/h]则均更高,并且差异均有统计学意义(Z=2.90、P=0.003,Z=-2.55、P=0.010,t=-4.22、P<0.001)。2组患儿抗双链(dsDNA)抗体滴度、补体3和4水平及肾外器官受累发生率分别比较,差异均无统计学意义(P>0.05)。研究组患儿尿蛋白水平较对照组显著增高,并且差异有统计学意义(Z=-2.11,P=0.034)。2组患儿脓尿、肉眼血尿、肾功能不全发生率,血清肌酐(SCr)、血尿素氮(BUN)、估算肾小球滤过率(eGFR)、肾组织病理学分型构成比分别比较,差异均无统计学意义(P>0.05)。与对照组相比,研究组患儿的肾小球坏死发生率、细胞和纤维性新月体占比均显著增高,并且差异均有统计学意义(P=0.012,Z=-2.16、P=0.030)。③对研究组和对照组患儿分别进行中位随访时间为42.0个月(21.5,63.5个月)与16.5个月(6.0,36.8个月)的随访结果显示,2组LN患儿达完全缓解(CR)时间分别为2.5个月(2.0,5.8个月)与4.5个月(2.3,6.8个月),并且差异无统计学意义(Z=-1.37,P=0.185)。2组患儿治疗6、12个月时CR率,LN复发率和终末期肾病(ESKD)进展率分别比较,差异均无统计学意义(P>0.05)。2组患儿持续CR和生存(未进展至ESKD)曲线分别比较,差异亦均无统计学意义(P>0.05)。

结论

与血清ANCA呈阴性LN患儿相比,血清ANCA呈阳性者的LN活动度更高,贫血程度更著,肾损害更严重,需及时诊断与治疗。但是,血清ANCA与LN患儿远期预后关系迄今尚未阐明。

Objective

To explore the clinical manifestations, renal histopathological features, and prognosis of pediatric lupus nephritis (LN) patients with positive serum antineutrophil cytoplasmic antibody (ANCA).

Methods

A total of 53 newly diagnosed LN children who were hospitalized in the Department of Nephrology, Capital Center for Children′s Health, Capital Medical University between January 2015 and February 2024 were selected. Based on the serum ANCA results, the children were divided into study group (n=9, with positive serum ANCA) and control group (n=44, with negative serum ANCA). A retrospective analysis was conducted to collect clinical data on the two groups, including clinical manifestations, laboratory test results, renal histopathological characteristics, and prognosis. Statistical comparisons were performed using independent samples t-test, Mann-Whitney U test, chi-square test, and Fisher′s exact test. This study was approved by the Ethics Committee of the Capital Center for Children′s Health, Capital Medical University (Approval No. SHERLL2021052).

Results

①Among 53 children with LN, 19 were male and 34 were female, with onset age of (11.2±2.8) years. There were no statistically significant differences in general clinical data such as gender ratio and age of onset between the study group and control group (P>0.05). ②Compared with the control group, the study group had lower levels of hemoglobin (Hb) [79.0 g/L (68.0, 88.5 g/L) vs 102.0 g/L (84.3, 113.8 g/L), Z=2.90, P=0.003], and higher systemic lupus erythematosus disease activity index 2000 (SLEDAI-2000) scores [23.0 points (18.0, 30.0 points) vs 18.0 points (16.0, 20.8 points), Z=-2.55, P=0.010], as well as higher erythrocyte sedimentation rate (ESR) [(86.1±43.5) mm/h vs (38.7±27.7) mm/h, t=-4.22, P<0.001]. There were no statistically significant differences in anti double stranded DNA (dsDNA) antibody titers, complement 3 and 4 levels, and incidence of extrarenal organ involvement between the two groups (P>0.05). The study group had higher levels of urine protein, and the difference was statistically significant (Z=-2.11, P=0.034). There were no statistically significant differences between the two groups in pyuria, gross hematuria, incidence of renal insufficiency, serum creatinine (SCr), blood urea nitrogen (BUN), estimated glomerular filtration rate (eGFR), and the distribution of renal pathological classification (P>0.05). Compared with the control group, the study group had a higher incidence of glomerular necrosis and a higher proportion of cells and cellular fibrous crescents (P=0.012; Z=-2.16, P=0.030). ③The median follow-up time for the study group and control group were 42.0 months (21.5, 63.5 months) and 16.5 months (6.0, 36.8 months), respectively. The complete remission (CR) time in the study group and control group were 2.5 months (2.0, 5.8 months) and 4.5 months (2.3, 6.8 months), respectively, and the difference was not statistically significant (Z=-1.37, P=0.185), and there were no statistically significant differences in the CR rate, the recurrence rate of LN, and the proportion of progress to end-stage renal disease (ESKD) (P>0.05). There were no statistically significant differences in the sustained CR and survival (non-progression to ESKD) curves between the two groups of children (P>0.05).

Conclusions

Compared with LN children with negative serum ANCA, LN children with positive serum ANCA have higher LN activity, more severe anemia and more serious renal damage, requiring timely diagnosis and treatment. However, the relationship between serum ANCA and the long-term prognosis of kidney disease in LN children remains unclear.

表1 研究组和对照组LN患儿SLEDAI-2000评分、基线免疫生化指标与肾外器官受累情况比较
表2 研究组和对照组患儿LN相关临床表现比较
表3 研究组和对照组LN患儿肾组织病理学特征相关指标比较
图1 研究组和对照组LN患儿持续CR和生存曲线比较注:研究组为血清ANCA结果呈阳性的LN患儿,对照组为血清ANCA结果呈阴性的LN患儿。图1B中的生存曲线、生存率分别指未进展至ESKD曲线、未进展至ESKD率。ANCA为抗中性粒细胞细胞质抗体,LN为狼疮性肾炎。CR为完全缓解,ESKD为终末期肾病
表4 研究组和对照组LN患儿随访时间、治疗方案及疗效比较
[1]
Oni L, Wright RD, Marks S, et al. Kidney outcomes for children with lupus nephritis[J]. Pediatr Nephrol, 2021, 36(6): 1377-1385. DOI: 10.1007/s00467-020-04686-1.
[2]
Windpessl M, Bettac EL, Gauckler P, et al. ANCA status or clinical phenotype: what counts more?[J]. Curr Rheumatol Rep, 2021, 23(6): 37. DOI: 10.1007/s11926-021-01002-0.
[3]
Folci M, Ramponi G, Solitano V, et al. Serum ANCA as disease biomarkers: clinical implications beyond vasculitis[J]. Clin Rev Allergy Immunol, 2022, 63(2): 107-123. DOI: 10.1007/s12016-021-08887-w.
[4]
Wang Y, Huang X, Cai J, et al. Clinicopathologic characteristics and outcomes of lupus nephritis with antineutrophil cytoplasmic antibody: a retrospective study[J]. Medicine (Baltimore), 2016, 95(4): e2580. DOI: 10.1097/MD.0000000000002580.
[5]
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus[J]. Arthritis Rheum, 1997, 40(9): 1725. DOI: 10.1002/art.1780400928.
[6]
Lewis EJ, Schwartz MM. Pathology of lupus nephritis[J]. Lupus, 2005, 14(1): 31-38. DOI: 10.1191/0961203305lu2056oa.
[7]
Bajema IM, Wilhelmus S, Alpers CE, et al. Revision of the International Society of Nephrology/Renal Pathology Society classification for lupus nephritis: clarification of definitions, and modified National Institutes of Health activity and chronicity indices[J]. Kidney Int, 2018, 93(4): 789-796. DOI: 10.1016/j.kint.2017.11.023.
[8]
Demir S, Gülhan B, Özen S, et al. Long-term renal survival of paediatric patients with lupus nephritis[J]. Nephrol Dial Transplant, 2022, 37(6): 1069-1077. DOI: 10.1093/ndt/gfab152.
[9]
Schwartz GJ, Muñoz A, Schneider MF, et al. New equations to estimate GFR in children with CKD[J]. J Am Soc Nephrol, 2009, 20(3): 629-637. DOI: 10.1681/ASN.2008030287.
[10]
Li Q, Song D, Wang F, et al. Clinicopathological characteristics and outcomes of Chinese patients with scanty immune deposits lupus nephritis: a large cohort study from a single center[J]. ScientificWorldJournal, 2014, 2014: 212597. DOI: 10.1155/2014/212597.
[11]
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. DOI: 10.3760/cma.j.issn.0578-1310.2018.02.003.
[12]
Sen D, Isenberg DA. Antineutrophil cytoplasmic autoantibodies in systemic lupus erythematosus[J]. Lupus, 2003, 12(9): 651-658. DOI: 10.1191/0961203303lu456rr.
[13]
Gajic-Veljic M, Lekic B, Nikolic M, et al. Level and avidity of antineutrophil cytoplasmic antibodies specific to lactoferrin are useful biomarkers in systemic lupus erythematosus[J]. Clin Rheumatol, 2022, 41(3): 709-720. DOI: 10.1007/s10067-021-05926-x.
[14]
Nakazawa D, Masuda S, Nishibata Y, et al. Neutrophils and NETs in kidney disease[J]. Nat Rev Nephrol, 2025, 21(6): 383-398. DOI: 10.1038/s41581-025-00944-3.
[15]
Said D, Rashad NM, Abdelrahmanc NS, et al. Antineutrophil cytoplasmic antibody in lupus nephritis: correlation with clinicopathological characteristics and disease activity[J]. Curr Rheumatol Rev, 202117(2): 213-221. DOI: 10.2174/1573397116999201208213422.
[16]
Wang S, Shang J, Xiao J, et al. Clinicopathologic characteristics and outcomes of lupus nephritis with positive antineutrophil cytoplasmic antibody[J]. Ren Fail, 2020, 42(1): 244-254. DOI: 10.1080/0886022X.2020.1735416.
[17]
Pyo JY, Jung SM, Song JJ, et al. ANCA positivity at the time of renal biopsy is associated with chronicity index of lupus nephritis[J]. Rheumatol Int, 2019, 39(5): 879-884. DOI: 10.1007/s00296-019-04263-2.
[18]
Turner-Stokes T, Wilson HR, Morreale M, et al. Positive antineutrophil cytoplasmic antibody serology in patients with lupus nephritis is associated with distinct histopathologic features on renal biopsy[J]. Kidney Int, 2017, 92(5): 1223-1231. DOI: 10.1016/j.kint.2017.04.029.
[19]
Dai G, Li L, Wang T, et al. Pulmonary involvement in children with systemic lupus erythematosus[J]. Front Pediatr, 2021, 8: 617137. DOI: 10.3389/fped.2020.617137.
[20]
Nisihara R, Vithoft G, Alencar I, et al. ANCA in patients with systemic lupus erythematosus. A cross sectional study in Brazilian patients and review of literature[J]. Lupus, 2024, 33(6): 574-586. DOI: 10.1177/09612033241240588.
[21]
Wang Y, Yu X, Xie X, et al. Clinical features and outcomes of patients with antineutrophil cytoplasmic antibody-positive systemic lupus erythematosus[J]. Ren Fail, 2023, 45(1): 2235431. DOI: 10.1080/0886022X.2023.2235431.
[22]
Gau CC, Tseng MH, Wu CY, et al. The impact of serum anti-neutrophil cytoplasmic antibody on clinical characteristics and outcomes in pediatric-onset systemic lupus erythematosus patients[J]. Front Med (Lausanne), 2021, 8: 647510. DOI: 10.3389/fmed.2021.647510.
[23]
Fan Y, Kang D, Chen Z, et al. Clinicopathological characteristics and outcomes of lupus nephritis patients with scanty immune depositions in kidney biopsies[J]. J Nephrol, 2023, 36(8): 2345-2354. DOI: 10.1007/s40620-023-01622-y.
[24]
Lacetera R, Calatroni M, Roggero L, et al. Prevalence and clinical significance of ANCA positivity in lupus nephritis: a case series of 116 patients and literature review[J]. J Nephrol, 2023, 36(4): 1059-1070. DOI: 10.1007/s40620-023-01574-3.
[25]
Li C, Wang JJ, Zhou ML, et al. Differences in clinico-pathological characteristics and outcomes between proteinase 3-ANCA positivity and myeloperoxidase-ANCA positivity in lupus nephritis[J]. Lupus, 2019, 28(9): 1111-1119. DOI: 10.1177/0961203319861680.
[26]
El-Garf K, El-Garf A, Gheith R, et al. A comparative study between the disease characteristics in adult-onset and childhood-onset systemic lupus erythematosus in Egyptian patients attending a large university hospital[J]. Lupus, 2021, 30(2): 211-218. DOI: 10.1177/0961203320972778.
[27]
Kang JH, Park DJ, Lee KE, et al. Comparison of clinical, serological, and prognostic differences among juvenile-,adult-,and late-onset lupus nephritis in Korean patients[J]. Clin Rheumatol, 2017, 36(6): 1289-1295. DOI: 10.1007/s10067-017-3641-6.
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