Anti-glomerular Basement Membrane Glomerulonephritis: A Study in Real Life

Frontiers in Medicine

article

Marina Sánchez-Agesta¹  Cristina Rabasco¹  María J. Soler²  Amir Shabaka³  Elisabeth Canllavi⁴  Saulo J. Fernández⁵  Juan M. Cazorla⁶  Esperanza López-Rubio⁷  Ana Romera⁸  Sergio Barroso⁹  Ana Huerta^1,10  Leonardo Calle^1,12  Milagros Sierra^1,13  Patricia Domínguez-Torres^1,14  Manuela Moreno-Ramírez^1,15  Sara Afonso^1,16  Victoria Mascarós^1,17  Armando Coca^1,18  Mario Espinosa¹ 

[1] Department of Nephrology, Hospital Universitario Reina Sofía;Department of Nephrology, Hospital Vall d’Hebron;Department of Nephrology, Hospital Clínico San Carlos;Department of Nephrology, Hospital 12 de Octubre;Department of Nephrology, Hospital Universitario Insular de Gran Canaria;Department of Nephrology, Hospital Universitario Puerta del Mar;Department of Nephrology, Hospital General de Albacete;Department of Nephrology, Hospital General Universitario de Ciudad Real;Department of Nephrology, Hospital Universitario de Badajoz;Department of Nephrology, Hospital Universitario Puerta de Hierro Majadahonda;RedinRen ISCIII RETYC 16/009, Hospital Universitario Puerta de Hierro Majadahonda;Department of Nephrology;Department of Nephrology, Hospital San Pedro;Department of Nephrology, Hospital Universitario Fundación Alcorcón;Department of Nephrology, Hospital Juan Ramón Jimenez;Department of Nephrology, Hospital Universitario La Paz;Department of Nephrology, Hospital Francesc Borja de Gandía;Department of Nephrology, Hospital Clínico Universitario de Valladolid

关键词: anti-glomerular basement membrane disease;    crescents;    glomerulonephritis (GN);    end-stage kidney disease (ESKD);    kidney survival;    plasma exchange;   

DOI  :  10.3389/fmed.2022.889185

学科分类：社会科学、人文和艺术（综合）

来源: Frontiers

【 摘 要 】

Introduction 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. Methods 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. Results 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% p 50% crescents (6 vs. 49%; p = 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); p 4.7 mg/dL and 50.5% crescents had an area under the curve (AUC) of 0.9 (95% CI 0.82–0.97; p 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.

【 授权许可】

CC BY