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 anti-neutrophil cytoplasmic antibody

作　　者：文思佳 陈丽植[1] 程程 林知朗 莫樱[1] 蒋小云[1] WEN Si-Jia;CHEN Li-Zhi;CHENG Cheng;LIN Zhi-Lang;MO Ying;JIANG Xiao-Yun(Children Kidney Disease Center,First Affiliated Hospital of Sun Yat-sen University,Guangzhou 510080,China)

机构地区：[1]中山大学附属第一医院儿科小儿肾病中心,广东广州510080

出　　处：《中国当代儿科杂志》2021年第1期55-60,共6页Chinese Journal of Contemporary Pediatrics

摘　　要：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。[中国当代儿科杂志,2021,23(1):55-60]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 ANCA-negative 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 ANCA-negative 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.[Chin J Contemp Pediatr,2021,23(1):55-60]

关 键 词：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 

分 类 号：R725.9[医药卫生—儿科]