Sánchez-Agesta Marina, Rabasco Cristina, Soler María J, Shabaka Amir, Canllavi Elisabeth, Fernández Saulo J, Cazorla Juan M, López-Rubio Esperanza, Romera Ana, Barroso Sergio, Huerta Ana, Calle Leonardo, Sierra Milagros, Domínguez-Torres Patricia, Moreno-Ramírez Manuela, Afonso Sara, Mascarós Victoria, Coca Armando, Espinosa Mario
Department of Nephrology, Hospital Universitario Reina Sofía, Córdoba, Spain.
Department of Nephrology, Hospital Vall d'Hebron, Barcelona, Spain.
Front Med (Lausanne). 2022 Jul 5;9:889185. doi: 10.3389/fmed.2022.889185. eCollection 2022.
Anti-glomerular basement membrane (anti-GBM) disease is a severe entity with few therapeutic options including plasma exchange and immunosuppressive agents. The aim of this study was to analyze the clinical and pathological features that predict the evolution of end-stage kidney disease (ESKD) and the kidney survival in a cohort of patients with anti-GBM disease with renal involvement in real life.
A retrospective multicentre observational study including 72 patients from 18 nephrology departments with biopsy-proven anti-GBM disease from 1999 to 2019 was performed. Progression to ESKD in relation to clinical and histological variables was evaluated.
Creatinine at admission was 8.6 (± 4) mg/dL and 61 patients (84.7%) required dialysis. Sixty-five patients (90.3%) underwent plasma exchange. Twenty-two patients (30.6%) presented pulmonary hemorrhage. Kidney survival was worse in patients with creatinine levels > 4.7 mg/dL (3 vs. 44% < 0.01) and in patients with > 50% crescents (6 vs. 49%; = 0.03). Dialysis dependence at admission and creatinine levels > 4.7 mg/dL remained independent significant predictors of ESKD in the multivariable analysis [HR (hazard ratio) 3.13 (1.25-7.84); HR 3 (1.01-9.14); < 0.01]. The discrimination value for a creatinine level > 4.7 mg/dL and 50.5% crescents had an area under the curve (AUC) of 0.9 (95% CI 0.82-0.97; < 0.001) and 0.77 (95% CI 0.56-0.98; = 0.008), respectively. Kidney survival at 1 and 2 years was 13.5 and 11%, respectively. Patient survival at 5 years was 81%.
In real life, patients with severe anti-GBM disease (creatinine > 4.7 mg/dL and > 50% crescents) remained with devastating renal prognosis despite plasma exchange and immunosuppressive treatment. New therapies for the treatment of this rare renal disease are urgently needed.
抗肾小球基底膜（anti-GBM）病是一种严重疾病，治疗选择有限，包括血浆置换和免疫抑制剂。本研究的目的是分析在现实生活中一组有肾脏受累的抗GBM病患者中，预测终末期肾病（ESKD）进展和肾脏生存率的临床和病理特征。
进行了一项回顾性多中心观察性研究，纳入了1999年至2019年来自18个肾脏病科的72例经活检证实为抗GBM病的患者。评估了与临床和组织学变量相关的ESKD进展情况。
入院时肌酐水平为8.6（±4）mg/dL，61例患者（84.7%）需要透析。65例患者（90.3%）接受了血浆置换。22例患者（30.6%）出现肺出血。肌酐水平>4.7mg/dL（3%对44%；P<0.01）和新月体>50%（6%对49%；P=0.03）的患者肾脏生存率较差。多变量分析显示，入院时依赖透析和肌酐水平>4.7mg/dL仍然是ESKD的独立显著预测因素[风险比（HR）3.13（1.25 - 7.84）；HR 3（1.01 - 9.14）；P<0.01]。肌酐水平>4.7mg/dL和新月体50.5%的判别值的曲线下面积（AUC）分别为0.9（95%CI 0.82 - 0.97；P<0.001）和0.77（95%CI 0.56 - 0.98；P = 0.008）。1年和2年的肾脏生存率分别为13.5%和11%。5年的患者生存率为81%。
在现实生活中，尽管进行了血浆置换和免疫抑制治疗，但严重抗GBM病（肌酐>4.7mg/dL和新月体>50%）患者的肾脏预后仍然很差。迫切需要针对这种罕见肾病的新疗法。
