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儿童抗中性粒细胞胞浆抗体阳性的IgA血管炎的临床特征

[Clinical features of IgA vasculitis with positive antineutrophil cytoplasmic antibody in children].

作者信息

Wan J L, Li P, Tan L W, Jiao J, Yang Q, Zhong C, Zhang G F, Yang H P, Li Q, Wang M

机构信息

Department of Nephrology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Pediatric Metabolism and Inflammatory Disease, Chongqing Municipal Health Commission Key Laboratory of Children's Vital Organ Development and Diseases, Chongqing 400014, China.

出版信息

Zhonghua Er Ke Za Zhi. 2025 Sep 2;63(9):972-979. doi: 10.3760/cma.j.cn112140-20250505-00385.

Abstract

To analyze the clinical features and risk factors for renal injury in children with antineutrophil cytoplasmic antibody (ANCA)-positive IgA vasculitis (IgAV). A case-control study was conducted. Seventy-two ANCA-positive IgAV children hospitalized at the Children's Hospital of Chongqing Medical University from January 2017 to October 2022 were enrolled as the ANCA-positive group. Propensity score matching (1∶4) using the nearest neighbor was performed with age and gender as covariate, and 288 cases ANCA-negative IgAV children were included as the ANCA-negative group. Patients with renal injury were named ANCA-positive IgAV nephritis (IgAVN) group and ANCA-negative IgAVN group, respectively. The ANCA-positive IgAVN group was further divided into myeloperoxidase (MPO) group and proteinase 3 (PR3) group based on the type of ANCA. Clinical data including manifestations, laboratory tests, renal injury, and prognosis were collected. Comparisons between groups were performed using independent sample -tests, Mann-Whitney tests, tests, or Fisher's exact tests. Kaplan-Meier curves were used to assess differences in the time to renal injury onset, and multivariate logistic regression was performed to identify independent risk factors for renal injury. Among the 72 ANCA-positive IgAV children (41 males, 31 females, age of 7.7 (5.3, 11.2) years), no significant difference in age or gender was observed compared to the ANCA-negative group (both >0.05). The ANCA-positive group had higher IgM levels, a higher incidence of recurrent rash, and shorter thrombin time (all <0.05). Among children with renal injury, the ANCA-positive group showed significant differences in the incidence of hematuria, clinical classification, and grade A prognosis compared to the ANCA-negative group (all 0.05), but no difference was found in the time to renal involvement onest or renal pathology (all 0.05). The MPO group had higher rates of microscopic hematuria, gross hematuria, acute renal insufficiency, glomerular sclerosis, and grade B prognosis compared to the ANCA-negative IgAVN group (all 0.05), with a later onset of renal involvement (0.05). Elevated serum creatinine (=1.08, 95% 1.03-1.14) and shortened thrombin time (=0.71, 95% 0.55-0.92) were independent risk factors for renal injury in ANCA-positive IgAV children (all <0.05). Children with ANCA-positive IgAV are more likely to experience recurrent rash. MPO-ANCA-positive IgAVN children have higher risks of hematuria, acute kidney injury and glomerular sclerosis, with later-onset but poorer renal prognosis compared to ANCA-negative IgAVN children. Higher serum creatinine levels and shorter thrombin time may be associated with renal injury in children with ANCA-positive IgAV.

摘要

分析抗中性粒细胞胞浆抗体(ANCA)阳性的IgA血管炎(IgAV)患儿肾损伤的临床特征及危险因素。进行了一项病例对照研究。将2017年1月至2022年10月在重庆医科大学附属儿童医院住院的72例ANCA阳性的IgAV患儿纳入ANCA阳性组。以年龄和性别作为协变量,采用最近邻倾向评分匹配法(1∶4),纳入288例ANCA阴性的IgAV患儿作为ANCA阴性组。有肾损伤的患者分别命名为ANCA阳性IgA血管炎肾炎(IgAVN)组和ANCA阴性IgAVN组。根据ANCA类型,将ANCA阳性IgAVN组进一步分为髓过氧化物酶(MPO)组和蛋白酶3(PR3)组。收集包括临床表现、实验室检查、肾损伤及预后等临床资料。组间比较采用独立样本t检验、Mann-Whitney检验、χ²检验或Fisher确切概率法。采用Kaplan-Meier曲线评估肾损伤发病时间的差异,并进行多因素logistic回归分析以确定肾损伤的独立危险因素。在72例ANCA阳性的IgAV患儿中(男41例,女31例,年龄7.7(5.3,11.2)岁),与ANCA阴性组相比,年龄和性别差异均无统计学意义(均>0.05)。ANCA阳性组IgM水平更高,皮疹复发率更高,凝血酶时间更短(均<0.05)。在有肾损伤的患儿中,与ANCA阴性组相比,ANCA阳性组血尿发生率、临床分型及A级预后差异均有统计学意义(均<0.05),但肾受累发病时间及肾病理差异均无统计学意义(均>0.05)。与ANCA阴性IgAVN组相比,MPO组镜下血尿、肉眼血尿、急性肾功能不全、肾小球硬化及B级预后发生率更高(均<0.05),肾受累发病时间更晚(<0.05)。血清肌酐升高(β=1.08,95%CI 1.03 - 1.14)和凝血酶时间缩短(β=0.71,95%CI 0.55 - 0.92)是ANCA阳性IgAV患儿肾损伤的独立危险因素(均<0.05)。ANCA阳性的IgAV患儿更易出现皮疹复发。与ANCA阴性IgAVN患儿相比,MPO-ANCA阳性的IgAVN患儿血尿、急性肾损伤及肾小球硬化风险更高,肾受累发病时间晚但肾预后更差。血清肌酐水平升高和凝血酶时间缩短可能与ANCA阳性IgAV患儿的肾损伤有关。

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