Objectives Integrating clinical and histological parameters into prognostic scores may enhance the prediction of progression to kidney failure in anti-neutrophil cytoplasm antibodies-associated vasculitis (AAV). This study aimed to evaluate the prognostic performance of histological classifications and scoring systems for kidney survival in AAV. Methods This retrospective cohort study included 101 AAV patients with kidney involvement diagnosed by biopsy and followed for ≥12 months. The main outcome was the time to kidney failure. The prognostic performance of each histological and prognostic score was evaluated using Harrell’s C statistic and Akaike’s Information Criteria. Results Among the 101 patients, 37 progressed to kidney failure over a median follow-up of 75 months (IQR 39–123). The Harrell’s C statistic was 0.702 (0.620–0.784), 0.606 (0.473–0.738), 0.801 (0.736–0.867), 0.782 (0.706–0.858), and 0.817 (0.749–0.885) for the EUVAS/Berden classification, Mayo Clinic Chronicity Score, Percentage of ANCA Crescentic Score (PACS), ANCA renal risk score (ARRS), and the improved ANCA kidney risk score (AKRiS), respectively. The AKRiS best discriminated the risk of kidney failure progression among subgroups. The AKRiS performance decreased with longer follow-up intervals. Adding the peak estimated glomerular filtration rate attained post-therapy improved the AKRiS performance at all follow-up intervals. Kidney relapses precipitated kidney failure in 71% of cases that progressed after the first year of follow-up. Conclusion The novel AKRiS enhances the prediction of kidney failure in AAV with kidney involvement. As the prognostic yield of AKRiS decreases over time, a second calculation of AKRiS, including post-therapy kidney function, may improve its long-term performance.

中文翻译：

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ANCA 相关血管炎肾脏存活的临床和组织学预后评分的表现


目的 将临床和组织学参数整合到预后评分中可以增强抗中性粒细胞胞质抗体相关性血管炎 (AAV) 进展为肾衰竭的预测。本研究旨在评估 AAV 肾脏存活的组织学分类和评分系统的预后表现。方法 这项回顾性队列研究纳入了 101 名经活检诊断为肾脏受累的 AAV 患者，并随访≥12 个月。主要结果是肾衰竭的时间。使用 Harrell 的 C 统计量和 Akaike 的信息标准评估每个组织学和预后评分的预后表现。结果 在 101 名患者中，中位随访 75 个月后，37 名患者进展为肾衰竭（IQR 39-123）。 EUVAS/Berden 分类的 Harrell C 统计量为 0.702 (0.620–0.784)、0.606 (0.473–0.738)、0.801 (0.736–0.867)、0.782 (0.706–0.858) 和 0.817 (0.749–0.885)， o 临床慢性病分别为评分、ANCA 新月评分 (PACS) 百分比、ANCA 肾脏风险评分 (ARRS) 和改进的 ANCA 肾脏风险评分 (AKRiS)。 AKRiS 最好地区分亚组中肾衰竭进展的风险。 AKRiS 性能随着随访间隔的延长而下降。添加治疗后达到的估计峰值肾小球滤过率可改善所有随访时间间隔的 AKRiS 性能。在第一年随访后进展的病例中，71% 的肾脏复发会导致肾衰竭。结论 新型 AKRiS 增强了肾脏受累 AAV 肾衰竭的预测。由于 AKRiS 的预后效果会随着时间的推移而降低，因此对 AKRiS 进行第二次计算（包括治疗后肾功能）可能会改善其长期表现。