OBJECTIVE Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS). METHODS Pediatric AAV patients from 11 referral centers in Turkey had been included to the study. The demographics, clinical findings, AAV subtypes, outcomes, and FFS were evaluated retrospectively. Kidney biopsies were classified histopathologically. RESULTS Totally, 39 patients were enrolled in the study. Among all patients, 74.4% had renal involvement, 56.4% ear-throat-nose involvement, and 51.3% had musculoskeletal involvement. Proteinase 3 (PR3)-ANCA was positive in 48.7%, and myeloperoxidase (MPO)-ANCA was positive in 30.8%. 69.2% of patients had impaired renal function, and 28.2% had progressed to end-stage renal disease (ESRD) during the follow-up. At the time of diagnosis, FFS was ≥ 2 in 53.8%. The most common histopathologic classifications were as follows: crescentic type in 40.7% and sclerotic type in 25.9%. Gastrointestinal and renal involvement, MPO-ANCA positivity, serum creatinine levels, and impaired renal function during the follow-up were significantly higher in patients with FFS ≥ 2, compared to patients with FFS < 2. Patients with FFS ≥ 2 had more common crescentic, mixed and sclerotic histopathologic findings in biopsies. By logistic regression analysis forward method, the strongest single-risk factor among all the parameters was the initial level of creatinine in patients with ESRD, compared to the other patients (p = 0,007). CONCLUSIONS Evaluation of the FFS, ANCA serology, and the creatinine levels may help to predict renal prognosis.

中文翻译：

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影响儿童ANCA相关血管炎的肾脏预后的临床和组织病理学预后因素。

目的抗中性粒细胞胞浆抗体（ANCA）相关的血管炎（AAV）在儿童中非常罕见，发病和死亡的风险增加。我们旨在从ANCA血清型，组织病理学分类和五因素评分（FFS）的角度评估儿童AAV的肾脏预后因素。方法将来自土耳其11个转诊中心的小儿AAV患者纳入研究。回顾性评估人口统计学，临床发现，AAV亚型，结局和FFS。肾脏活检组织病理学分类。结果总共有39名患者被纳入研究。在所有患者中，肾脏受累占74.4％，耳鼻喉受累占56.4％，肌肉骨骼受累占51.3％。蛋白酶3（PR3）-ANCA阳性率为48.7％，髓过氧化物酶（MPO）-ANCA阳性率为30.8％。随访期间，有69.2％的患者肾功能受损，有28.2％的患者发展为晚期肾病（ESRD）。诊断时FFS≥2的占53.8％。最常见的组织病理学分类如下：新月型占40.7％，硬化型占25.9％。与FFS <2的患者相比，FFS≥2的患者在随访期间胃肠道和肾脏受累，MPO-ANCA阳性，血清肌酐水平和肾功能受损显着高于FFS <2的患者。活检中的混合，硬化和硬化组织病理学发现。通过Logistic回归分析正向方法，所有参数中最强的单一风险因素是ESRD患者的肌酐初始水平，与其他患者相比（p = 0,007）。结论对FFS，ANCA血清学和肌酐水平的评估可能有助于预测肾脏的预后。