Anti-glomerular Basement Membrane Glomerulonephritis: A Study in Real Life
Por:
Sanchez-Agesta, M, Rabasco, C, Soler, M, Shabaka, A, Canllavi, E, Fernandez, S, Cazorla, J, Lopez-Rubio, E, Romera, A, Barroso, S, Huerta, A, Calle, L, Sierra, M, Dominguez-Torres, P, Moreno-Ramirez, M, Afonso, S, Mascaros, V, Coca, A and Espinosa, M
Publicada:
5 jul 2022
Categoría:
Medicine (miscellaneous)
Resumen:
IntroductionAnti-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. MethodsA 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. ResultsCreatinine 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 < 0.01) and in patients with > 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 < 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; p < 0.001) and 0.77 (95% CI 0.56-0.98; p = 0.008), respectively. Kidney survival at 1 and 2 years was 13.5 and 11%, respectively. Patient survival at 5 years was 81%. ConclusionIn 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.
Filiaciones:
Sanchez-Agesta, M:
Hosp Univ Reina Sofia, Dept Nephrol, Cordoba, Spain
Rabasco, C:
Hosp Univ Reina Sofia, Dept Nephrol, Cordoba, Spain
Soler, M:
Hosp Valle De Hebron, Dept Nephrol, Barcelona, Spain
Shabaka, A:
Hosp Clin San Carlos, Dept Nephrol, Madrid, Spain
Canllavi, E:
Hosp 12 Octubre, Dept Nephrol, Madrid, Spain
Fernandez, S:
Hosp Univ Insular Gran Canaria, Dept Nephrol, Las Palmas Gran Canaria, Spain
Cazorla, J:
Hosp Univ Puerta Mar, Dept Nephrol, Cadiz, Spain
Lopez-Rubio, E:
Hosp Gen Albacete, Dept Nephrol, Albacete, Spain
Romera, A:
Hosp Gen Univ Ciudad Real, Dept Nephrol, Ciudad Real, Spain
Barroso, S:
Hosp Univ Badajoz, Dept Nephrol, Badajoz, Spain
Huerta, A:
Hosp Univ Puerta Hierro Majadahonda, Dept Nephrol, Madrid, Spain
Hosp Univ Puerta Hierro Majadahonda, RedinRen ISCIII RETYC 16 009, Madrid, Spain
Calle, L:
Complejo Asistencial Segovia, Dept Nephrol, Segovia, Spain
Sierra, M:
Hosp San Pedro, Dept Nephrol, Logrono, Spain
Dominguez-Torres, P:
Hosp Univ Fdn Alcorcon, Dept Nephrol, Madrid, Spain
Moreno-Ramirez, M:
Hosp Juan Ramon Jimenez, Dept Nephrol, Huelva, Spain
Afonso, S:
Hosp Univ La Paz, Dept Nephrol, Madrid, Spain
:
Hosp Francesc Borja Gandia, Dept Nephrol, Valencia, Spain
Coca, A:
Univ Valladolid, Hosp Clin, Dept Nephrol, Valladolid, Spain
Espinosa, M:
Hosp Univ Reina Sofia, Dept Nephrol, Cordoba, Spain
Green Published, gold
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