Abstract：
The aim of this study was to evaluate the clinical features, pathological characteristics, and prognosis in myeloperoxidase (MPO)-antineutrophil cytoplasmic antibodies (ANCA)-associated glomerulonephritis (AAGN) with renal arteritis. The study involved 97 children from five pediatric clinical centers with MPO-AAGN who exhibited distinct clinical features. The patients were divided into AAGN-A+ and AAGN-A-, based on the presence or absence of arteritis, and the disparities in clinical, histopathological characteristics, and prognosis between the two groups was evaluated. In contrast to the AAGN-A- group, the children in the AAGN-A+ group exhibited more pronounced clinical symptoms and renal pathological injury. Arteritis positively moderately correlated with the serum creatinine, interleukin-6, urinary neutrophil gelatinase-associated lipocalin, negatively moderately correlated with serum complement C3. The renal survival rate in the AAGN-A+ group was significantly poorer than AAGN-A- group (χ2 = 4.278, p = 0.039). Arteritis showed a good predictive value for end-stage kidney disease (ESKD), and C3 deposition, ANCA renal risk score and arteritis were independent risk factors for the development of ESKD in children with MPO-AAGN. Arteritis is a significant pathological change observed in children with MPO-AAGN, and the formation of arteritis may be related to the inflammatory response and activation of the complement system.
摘要：
目的：本研究的目的是评估临床特征，髓过氧化物酶（MPO）-抗中性粒细胞胞浆抗体相关性肾小球肾炎（AAGN）伴肾动脉炎的病理特征和预后。
方法：该研究包括来自5个MPO-AAGN儿科临床中心的97名患儿，他们表现出不同的临床特征。将患者分为AAGN-A+和AAGN-A-,根据动脉炎的存在与否，以及临床上的差异，组织病理学特征，并对两组患者的预后进行评价。
结果：与AAGN-A组相比，AAGN-A+组患儿表现出更明显的临床症状和肾脏病理损伤。动脉炎与血清肌酐（Scr）呈中度正相关，IL-6(白介素-6),尿中性粒细胞明胶酶相关脂质运载蛋白（NGAL），与血清补体C3呈中度负相关。AAGN-A+组的肾脏生存率明显低于AAGN-A-组(χ2=4.278,P=0.039)。动脉炎对终末期肾病（ESKD）具有良好的预测价值，C3沉积和动脉炎是MPO-AAGN患儿发生ESKD的独立危险因素。
结论：动脉炎是MPO-AAGN患儿的显著病理变化，动脉炎的形成可能与炎症反应和补体系统的激活有关。
方法：该研究包括来自5个MPO-AAGN儿科临床中心的97名患儿，他们表现出不同的临床特征。将患者分为AAGN-A+和AAGN-A-,根据动脉炎的存在与否，以及临床上的差异，组织病理学特征，并对两组患者的预后进行评价。
结果：与AAGN-A组相比，AAGN-A+组患儿表现出更明显的临床症状和肾脏病理损伤。动脉炎与血清肌酐（Scr）呈中度正相关，IL-6(白介素-6),尿中性粒细胞明胶酶相关脂质运载蛋白（NGAL），与血清补体C3呈中度负相关。AAGN-A+组的肾脏生存率明显低于AAGN-A-组(χ2=4.278,P=0.039)。动脉炎对终末期肾病（ESKD）具有良好的预测价值，C3沉积和动脉炎是MPO-AAGN患儿发生ESKD的独立危险因素。
结论：动脉炎是MPO-AAGN患儿的显著病理变化，动脉炎的形成可能与炎症反应和补体系统的激活有关。
