Renal Division, Peking University First Hospital, Beijing, China,
Institute of Nephrology, Peking University, Beijing, China,
Am J Nephrol. 2022;53(5):397-406. doi: 10.1159/000523713. Epub 2022 Apr 22.
Anti-glomerular basement membrane (GBM) disease is a rare but the most aggressive form of glomerulonephritis. To dissect the prognostic factors, we retrospectively analyzed the clinical features of a large cohort and compared the clinical features and prognosis during decades.
Data on clinical manifestation, treatment, and prognosis were collected. Cox models and receiver operating characteristic (ROC) curve were used to investigate the predictors for outcomes. The Kaplan-Meier curve and log-rank test were used to compare kidney and patient survival.
A total of 448 patients were enrolled. Patient survival and kidney survival at 1 year was 69.4% and 37.7%, respectively. During the past 3 decades, mortality at 3 months and 1 year significantly dropped from 37.5% and 57.1% in 1991-2000 to 2.8% and 6.9% in 2011-2020 (p < 0.001), respectively; kidney prognosis showed a tendency of improvement as well. Serum creatinine (Scr) on diagnosis (HR, 1.16; 95% CI, 1.05-1.29) and crescent percentage (HR, 1.73; 95% CI, 1.34-2.24) were independent predictors for end-stage kidney disease. ROC curve showed that the optimal cutoff point of Scr on diagnosis for prediction of dialysis dependency at 1 year was 536.4 μmol/L (sensitivity 88.3% and specificity 80.8%). Antineutrophil cytoplasmic antibodies (ANCAs) positivity (HR, 4.43; 95% CI, 1.72-11.38) was a predictor for mortality. Plasma exchange was associated with a better patient prognosis (HR, 0.40; 95% CI 0.16-0.95).
Scr on diagnosis and percentage of crescents were predictors for kidney outcomes. Positive ANCA was a predictor for mortality. Overall patient prognosis of anti-GBM disease was improved during the past 3 decades.
抗肾小球基底膜 (GBM) 病是一种罕见但最具侵袭性的肾小球肾炎。为了剖析预后因素，我们回顾性分析了一个大样本队列的临床特征，并比较了过去几十年的临床特征和预后。
收集临床表现、治疗和预后的数据。使用 Cox 模型和受试者工作特征 (ROC) 曲线来研究结局的预测因素。使用 Kaplan-Meier 曲线和对数秩检验来比较肾脏和患者的生存情况。
共纳入 448 例患者。患者和肾脏的 1 年生存率分别为 69.4%和 37.7%。在过去的 30 年中，3 个月和 1 年的死亡率从 1991-2000 年的 37.5%和 57.1%显著下降到 2011-2020 年的 2.8%和 6.9%（p<0.001）；肾脏预后也呈改善趋势。诊断时的血清肌酐（Scr）（HR，1.16；95%CI，1.05-1.29）和新月体百分比（HR，1.73；95%CI，1.34-2.24）是终末期肾病的独立预测因素。ROC 曲线显示，诊断时 Scr 预测 1 年内依赖透析的最佳截断点为 536.4μmol/L（灵敏度 88.3%，特异性 80.8%）。抗中性粒细胞胞质抗体（ANCA）阳性（HR，4.43；95%CI，1.72-11.38）是死亡的预测因素。血浆置换与患者预后较好相关（HR，0.40；95%CI 0.16-0.95）。
诊断时的 Scr 和新月体百分比是肾脏结局的预测因素。ANCA 阳性是死亡的预测因素。在过去的 30 年中，抗 GBM 病患者的整体预后得到了改善。
