Kang Zhijuan, Xun Mai, Li Zhihui, Yang Zuocheng
Department of Pediatrics, Third Xiangya Hospital of Central South University, Changsha, China.
Department of Nephrology, Rheumatology and Immunology, Hunan Children's Hospital, Changsha, China.
Front Pediatr. 2022 Oct 21;10:959212. doi: 10.3389/fped.2022.959212. eCollection 2022.
IgA vasculitis (IgAV) combined with nephrotic-range proteinuria is uncommon, and nephrotic-range proteinuria is considered a risk factor for poor prognosis in children with IgAV. There are few clinical studies with large samples.
Children with IgAV and nephrotic-range proteinuria who were hospitalized at the Department of Nephrology, Rheumatology and Immunology, Hunan Children's Hospital, from March 2008 to January 2020 were retrospectively studied; the patients were aged ≤18 years and were followed up for ≥12 months. We analyzed clinical characteristics, pathological changes, treatment responses, and their relationships in patients with IgAV combined with nephrotic-range proteinuria.
Two hundred seventy-seven children with an average age at onset of IgAV with nephritis (IgAVN) of 8.0 years (interquartile range (IQR), 6.0-10.0) were enrolled; 65.7% were aged 6-10 years. The male-to-female ratio was 1.35:1. All children had both nephrotic-range proteinuria and hematuria, 49 (17.7%) had hypoalbuminemia, and 9 (3.2%) had estimated glomerular filtration rate < 90 (mL/min/1.73 m). All included children were followed up for at least 1 year. At 3, 6, and 12 months of follow-up, the remission rates of proteinuria in children with IgAV combined with nephrotic-range proteinuria were 27.8%, 62.1%, and 83.0%, respectively, and the remission rates of hematuria were 1.4%, 8.7%, and 35.7%, respectively. In addition, children with age at onset of IgAV with nephrotic-range proteinuria ≥10 years, who were female, who had proteinuria ≥100 mg/kg/24 h, and who had a pathological grade III or above had lower remission rates of hematuria and proteinuria (< 0.05). Multivariate factor analysis was performed by logistic regression and showed age at onset of IgAVN ≥ 10 years and crescents to be risk factors for nonremission of proteinuria at 12 months of follow-up (< 0.05).
Age at onset of IgAVN, sex, proteinuria level, pathological grade, and crescents significantly affect proteinuria remission in children with IgAV combined with nephrotic-range proteinuria.
IgA 血管炎（IgAV）合并肾病范围蛋白尿并不常见，且肾病范围蛋白尿被认为是 IgAV 患儿预后不良的危险因素。大型样本的临床研究较少。
对 2008 年 3 月至 2020 年 1 月在湖南省儿童医院肾病、风湿免疫科住院的 IgAV 合并肾病范围蛋白尿患儿进行回顾性研究；患者年龄≤18 岁，随访时间≥12 个月。我们分析了 IgAV 合并肾病范围蛋白尿患者的临床特征、病理变化、治疗反应及其相互关系。
共纳入 277 例 IgA 血管炎伴肾炎（IgAVN）患儿，平均发病年龄为 8.0 岁（四分位间距（IQR），6.0 - 10.0）；65.7%的患儿年龄在 6 - 10 岁。男女比例为 1.35:1。所有患儿均有肾病范围蛋白尿和血尿，49 例（17.7%）有低蛋白血症，9 例（3.2%）估计肾小球滤过率＜90（mL/min/1.73m²）。所有纳入患儿均随访至少 1 年。在随访的 3、6 和 12 个月时，IgAV 合并肾病范围蛋白尿患儿的蛋白尿缓解率分别为 27.8%、62.1%和 83.0%，血尿缓解率分别为 1.4%、8.7%和 35.7%。此外，IgAV 合并肾病范围蛋白尿发病年龄≥10 岁、女性、蛋白尿≥100mg/kg/24h 且病理分级为 III 级及以上的患儿血尿和蛋白尿缓解率较低（P＜0.05）。采用逻辑回归进行多因素分析，结果显示 IgAVN 发病年龄≥10 岁和新月体是随访 12 个月时蛋白尿未缓解的危险因素（P＜0.05）。
IgAVN 的发病年龄、性别、蛋白尿水平、病理分级和新月体显著影响 IgAV 合并肾病范围蛋白尿患儿的蛋白尿缓解情况。
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