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. 2022 Jul 5:9:889185.
doi: 10.3389/fmed.2022.889185. eCollection 2022.

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

Marina Sánchez-Agesta  1 , Cristina Rabasco  1 , María J Soler  2 , Amir Shabaka  3 , Elisabeth Canllavi  4 , Saulo J Fernández  5 , Juan M Cazorla  6 , Esperanza López-Rubio  7 , Ana Romera  8 , Sergio Barroso  9 , Ana Huerta  10   11 , Leonardo Calle  12 , Milagros Sierra  13 , Patricia Domínguez-Torres  14 , Manuela Moreno-Ramírez  15 , Sara Afonso  16 , Victoria Mascarós  17 , Armando Coca  18 , Mario Espinosa  1 ; Spanish Group for the Study of Glomerular Diseases (GLOSEN)
Affiliations

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

Marina Sánchez-Agesta et al. Front Med (Lausanne). .

Abstract

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 < 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%.

Conclusion: 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.

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

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

FIGURE 1
FIGURE 1
The flowchart shows the patient’s outcomes according to the treatment received.
FIGURE 2
FIGURE 2
Actuarial renal survival by Kaplan–Meier curve according to the presence of > 50% of crescents (A), dialysis at presentation (B), and creatinine > 4.7 mg/dl (C).
FIGURE 3
FIGURE 3
The ROC curve for predicting end-renal stage disease development with creatinine al presentation > 4.7 mg/dl. AUC area under the curve, ROC curve receiver-operating characteristic curve.
FIGURE 4
FIGURE 4
The ROC curve for predicting end-renal stage disease development with > 50% of crescents. AUC area under the curve, ROC curve receiver-operating characteristic curve.
See this image and copyright information in PMC

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