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

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.

Estudio evolutivo de las glomerulonefritis en Castilla-La Mancha (GLOMANCHA) en el periodo 1994-2008 / Study of glomerulonephritis evolution in Castilla-La Mancha (GLOMANCHA) between 1994 and 2008

Antecedentes: Los registros de biopsias renales permiten la recogida de datos histopatológicos que, puestos en su conjunto, ayudan a comprender enfermedades y su historia natural. Objetivos: Analizar los datos del Registro de Glomerulonefritis de Castilla-La Mancha (GLOMANCHA) y la evolución de las diferentes enfermedades biopsiadas (1994-2008). Métodos: Se recogieron las 6 enfermedades biopsiadas más prevalentes durante los 14 años (941 biopsias) en 5 hospitales de la comunidad autónoma. En 2008 se evaluó la situación vital y renal de los pacientes y se analizaron los factores asociados a dicha situación en cada enfermedad. Resultados: De las 941 biopsias, el 59% fueron de varones con una edad media de 48±18 años. En el momento de la biopsia la mediana de filtrado glomerular (FG) era de 50,3 (25,5-76,3) ml/min/1,73 m2 y la de proteinuria de 3,4 (1,5-6,4) g al día. La enfermedad más frecuente fue la nefropatía lúpica, seguida de la glomeruloesclerosis focal y segmentaria, y de la membranosa. El mejor pronóstico renal durante el seguimiento (media 7,3±4,8 años) fue el de la nefropatía lúpica y la nefropatía por cambios mínimos; la glomeruloesclerosis focal y segmentaria y las glomerulonefritis rápidamente progresivas de tipo 3 tuvieron el peor pronóstico renal. Esta última, además, tuvo el peor pronóstico vital. Conclusiones: GLOMANCHA demuestra el mal pronóstico de las glomerulonefritis rápidamente progresivas de tipo 3, a diferencia de la nefropatía por cambios mínimos y lúpica. La función renal es un predictor independiente de supervivencia renal y de mortalidad en nuestra población (AU)

Background: Renal biopsy registries allow histopathological data to be collected to improve knowledge of different pathologies and their natural history. Aim: To analyse the data of the Castilla La Mancha Glomerulonephritis Registry (GLOMANCHA) and the evolution of the different biopsy-proven pathologies between 1994 and 2008. Methods: The 6 most common biopsy-proven pathologies were collected during the 14 years of the study (941 biopsies) in the 5 participant centres of the autonomous community. In 2008, we assessed patient renal survival and mortality and we evaluated associated factors to each situation for each pathology. Results: Of the 941 biopsies, 59% belonged to men, with a mean age of 48±18 years. At the time of the biopsy, the median glomerular filtration rate was 50.3 (25.5-76.3) ml/min/1,73 m2 and median proteinuria was 3.4 (1.5-6.4) grams per day. The most common pathology were lupus nephropathy, followed by focal segmental glomerulosclerosis and membranous nephropathy. Lupus nephropathy and minimal change disease achieved the best renal prognosis during follow-up (mean 7.3±4.8 years). Rapidly progressive glomerulonephritis type 3 and focal segmental glomerulosclerosis had the worst renal prognosis. In addition, rapidly progressive glomerulonephritis type 3 presented the worst vital prognosis. Conclusions: In GLOMANCHA, we demonstrate the poor prognosis of rapidly progressive glomerulonephritis type 3, in contrast to minimal change disease or lupus nephropathy. Renal function is an independent predictor of renal survival and mortality in this study (AU)

Study of glomerulonephritis evolution in Castilla-La Mancha (GLOMANCHA) between 1994 and 2008. / Estudio evolutivo de las glomerulonefritis en Castilla-La Mancha (GLOMANCHA) en el periodo 1994-2008.

BACKGROUND: Renal biopsy registries allow histopathological data to be collected to improve knowledge of different pathologies and their natural history. AIM: To analyse the data of the Castilla La Mancha Glomerulonephritis Registry (GLOMANCHA) and the evolution of the different biopsy-proven pathologies between 1994 and 2008. METHODS: The 6 most common biopsy-proven pathologies were collected during the 14 years of the study (941 biopsies) in the 5 participant centres of the autonomous community. In 2008, we assessed patient renal survival and mortality and we evaluated associated factors to each situation for each pathology. RESULTS: Of the 941 biopsies, 59% belonged to men, with a mean age of 48±18 years. At the time of the biopsy, the median glomerular filtration rate was 50.3 (25.5-76.3) ml/min/1,73 m(2) and median proteinuria was 3.4 (1.5-6.4) grams per day. The most common pathology were lupus nephropathy, followed by focal segmental glomerulosclerosis and membranous nephropathy. Lupus nephropathy and minimal change disease achieved the best renal prognosis during follow-up (mean 7.3±4.8 years). Rapidly progressive glomerulonephritis type 3 and focal segmental glomerulosclerosis had the worst renal prognosis. In addition, rapidly progressive glomerulonephritis type 3 presented the worst vital prognosis. CONCLUSIONS: In GLOMANCHA, we demonstrate the poor prognosis of rapidly progressive glomerulonephritis type 3, in contrast to minimal change disease or lupus nephropathy. Renal function is an independent predictor of renal survival and mortality in this study.