Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Department of Neurosurgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China.
Front Immunol. 2022 Apr 14;13:857813. doi: 10.3389/fimmu.2022.857813. eCollection 2022.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is relatively rare in children. This article aimed to analyze clinical and renal histology findings and different responses to induction treatment associated with the long-term renal outcomes in children with AAV in a single center.
All pediatric patients with AAV admitted to Tongji Hospital from January 2002 to January 2021 were included in the study. The demographic, clinical, pathological, laboratory, and treatment data and outcomes were collected and analyzed to identify predictors associated with response to induction treatment and progression to end-stage renal disease (ESRD).
In total, 48 children with AAV were included in this cohort; 81.25% of them were women, and 91.7% were microscopic polyangiitis (MPA). Kidney involvement was found in 45 patients (93.75%). The most common histopathological subtype was crescentic form in this cohort according to Berden's classification. In total, 34 patients (70.8%) showed eGFR <60 ml/min/1.73 m at the time of diagnosis. Complete and partial remission was achieved in 8 patients (16.7%) and 19 patients (39.6%), respectively, following 6-month induction treatment. Half of the patients eventually progressed to ESRD at a mean time of (13.04 ± 15.83) months after diagnosis. The independent predictors of nonremission following induction treatment and progression to ESRD were baseline eGFR <60 ml/min/1.73 m and hypertension at diagnosis. Renal survival significantly decreased over time in patients with renal sclerotic subtypes or those with nonremission following induction treatment by Kaplan-Meier curve estimation.
Our study demonstrates that women, MPA, and crescentic subtypes are predominant in pediatric AAV in China. Initial renal failure (eGFR <60 ml/min/1.73 m), hypertension, sclerotic pathological subtype, and nonremission following induction treatment are predictive of long-term renal outcomes.
抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）在儿童中相对少见。本文旨在分析单中心 48 例儿童 AAV 的临床和肾脏组织学表现，以及不同诱导治疗反应与长期肾脏结局的关系。
回顾性分析 2002 年 1 月至 2021 年 1 月期间在我院住院的所有儿童 AAV 患者的临床资料，分析其与诱导治疗反应和进展至终末期肾病（ESRD）相关的预测因素。
本研究共纳入 48 例儿童 AAV 患者，81.25%为女性，91.7%为显微镜下多血管炎（MPA）。45 例（93.75%）患者存在肾脏受累。根据 Berden 分类，该队列中最常见的组织病理学亚型为新月体性肾炎。诊断时，34 例（70.8%）患者的 eGFR<60ml/min/1.73m。6 个月诱导治疗后，8 例（16.7%）和 19 例（39.6%）患者达到完全缓解和部分缓解。平均（13.04±15.83）个月后，有 24 例（50.0%）患者最终进展为 ESRD。诱导治疗后未缓解和进展为 ESRD 的独立预测因素为基线 eGFR<60ml/min/1.73m 和诊断时的高血压。Kaplan-Meier 曲线估计显示，存在肾脏硬化性亚型或诱导治疗后未缓解的患者，其肾脏存活率随时间显著降低。
本研究表明，中国儿童 AAV 以女性、MPA 和新月体性肾炎为主。初始肾功能衰竭（eGFR<60ml/min/1.73m）、高血压、硬化性病理亚型和诱导治疗后未缓解是预测长期肾脏结局的因素。
