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伴抗中性粒细胞胞浆抗体阳性狼疮性肾炎患儿临床病理特征分析
文思佳, 陈丽植, 程程, 林知朗, 莫樱, 蒋小云
中国当代儿科杂志 ›› 2021, Vol. 23 ›› Issue (1) : 55-60.
PDF(1464 KB)
PDF(1464 KB)
伴抗中性粒细胞胞浆抗体阳性狼疮性肾炎患儿临床病理特征分析
Clinicopathological features of children with lupus nephritis with positive antineutrophil cytoplasmic antibody
目的 分析伴抗中性粒细胞胞浆抗体（ANCA）阳性狼疮性肾炎（LN）患儿的临床及病理特征。方法 回顾性选择2003年1月至2019年12月于中山大学附属第一医院诊断的LN患儿，依据血清ANCA结果分为ANCA阳性组（n=59）和ANCA阴性组（n=454），比较两组患儿临床表现、组织病理学、治疗缓解与预后情况。结果 ANCA阳性组白细胞降低并伴红细胞沉降率增快（P < 0.05）。两组患儿血肌酐、尿蛋白、尿红细胞差异无统计学意义（P > 0.05）。308例患儿行肾脏穿刺活检，光镜结果提示ANCA阳性组细胞纤维性新月体比例高于ANCA阴性组（P < 0.05），免疫复合物沉积比例低于ANCA阴性组（P < 0.05）。两组患儿治疗缓解率、生存情况差异无统计学意义（P > 0.05）。结论 ANCA阳性LN患儿肾脏病理损害更重，与临床表现不完全对应平行，及时行肾脏穿刺活检至关重要。
Objective To study the clinicopathological features of children with lupus nephritis (LN) with positive anti-neutrophil cytoplasmic antibody (ANCA). Methods A retrospective analysis was performed for the children who were diagnosed with LN in the First Affiliated Hospital of Sun Yat-sen University from January 2003 to December 2019. According to the results of serum ANCA, they were divided into two groups: ANCA-positive group (n=59) and ANCAnegative group (n=454). The two groups were compared in terms of clinical manifestations, histopathological features, remission rate, and prognosis. Results Compared with the ANCA-negative group, the ANCA-positive group had a significant reduction in leukocytes and a significant increase in erythrocyte sedimentation rate (P < 0.05). There were no significant differences between the two groups in serum creatinine, urine protein, and urine red blood cell count (P > 0.05). A total of 308 children underwent kidney biopsy. The results on light microscopy showed that compared with the ANCAnegative group, the ANCA-positive group had a significantly higher proportion of children with cellular fibrous crescents (P < 0.05) and a significantly lower proportion of children with immune complex deposition (P < 0.05). There were no significant differences between the two groups in the remission rate and survival rate (P > 0.05). Conclusions Children with ANCA-positive LN tend to have more severe renal pathological injury, which is not exactly parallel with clinical manifestations, suggesting that timely renal biopsy is of great importance.
系统性红斑狼疮 / 狼疮性肾炎 / 抗中性粒细胞胞浆抗体 / 临床病理特点 / 儿童
Systemic lupus erythematosus / Lupus nephritis / Anti-neutrophil cytoplasmic antibody / Clinicopathological feature / Child
[1] Malattia C, Martini A. Paediatric-onset systemic lupus erythematosus[J]. Best Pract Res Clin Rheumatol, 2013, 27(3):351-362.
[2] 蒋麒俊, 蔡枫. 系统性红斑狼疮患者血清中不典型ANCA抗体检测及临床意义[J]. 检验医学与临床, 2017, 14(24):3646-3648.
[3] Moiseev S, Cohen Tervaert JW, Arimura Y, et al. 2020 international consensus on ANCA testing beyond systemic vasculitis[J]. Autoimmun Rev, 2020, 19(9):102618.
[4] 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.
[5] 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.
[6] Brunner HI, Gladman DD, Ibañez D, et al. Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus[J]. Arthritis Rheum, 2008, 58(2):556-562.
[7] 杨晓, 姜红. 抗中性粒细胞胞浆抗体与小儿系统性红斑狼疮活动性及狼疮性肾炎相关性分析[J]. 中国实用儿科杂志, 2013, 28(10):784-786.
[8] Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus[J]. Arthritis Rheum, 1997, 40(9):1725.
[9] 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.
[10] Weening JJ, D'Agati VD, Schwartz MM, et al. The classification of glomerulonephritis in systemic lupus erythematosus revisited[J]. J Am Soc Nephrol, 2004, 15(2):241-250.
[11] Gladman DD, Ibañez D, Urowitz MB. Systemic lupus erythematosus disease activity index 2000[J]. J Rheumatol, 2002, 29(2):288-291.
[12] Austin HA 3rd, Muenz LR, Joyce KM, et al. Prognostic factors in lupus nephritis. Contribution of renal histologic data[J]. Am J Med, 1983, 75(3):382-391.
[13] Fanouriakis A, Kostopoulou M, Cheema K, et al. 2019 update of the joint European League against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of lupus nephritis[J]. Ann Rheum Dis, 2020, 79(6):713-723.
[14] Nasr SH, D'Agati VD, Park HR, et al. Necrotizing and crescentic lupus nephritis with antineutrophil cytoplasmic antibody seropositivity[J]. Clin J Am Soc Nephrol, 2008, 3(3):682-690.
[15] 李翠, 周敏林, 梁丹丹, 等. 血清抗中性粒细胞胞质抗体阳性狼疮性肾炎患者的临床病理特征及预后[J]. 肾脏病与透析肾移植杂志, 2017, 26(1):31-36.
[16] Wenderfer SE, Ruth NM, Brunner HI. Advances in the care of children with lupus nephritis[J]. Pediatr Res, 2017, 81(3):406-414.
[17] Davidson A, Aranow C, Mackay M. Lupus nephritis:challenges and progress[J]. Curr Opin Rheumatol, 2019, 31(6):682-688.
[18] Li C, Zhou ML, Liang DD, et al.Treatment and clinicopathological characteristics of lupus nephritis with antineutrophil cytoplasmic antibody positivity:a case-control study[J]. BMJ Open, 2017, 7(7):e015668.
[19] Masani NN, Imbriano LJ, D'Agati VD, et al. SLE and rapidly progressive glomerulonephritis[J]. Am J Kidney Dis, 2005, 45(5):950-955.
[20] 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.
[21] Hilhorst M, van Paassen P, Tervaert JW, et al. Proteinase 3-ANCA vasculitis versus myeloperoxidase-ANCA vasculitis[J]. J Am Soc Nephrol, 2015, 26(10):2314-2327.
