Predictors of renal and patient outcomes in anti-GBM disease: clinicopathologic analysis of a two-centre cohort.

Abstract

BACKGROUND:

Patients with anti-glomerular basement membrane (GBM) disease are at increased risk of morbidity and mortality from renal failure, pulmonary haemorrhage or complications of treatment. One-third also have circulating anti-neutrophil cytoplasmic antibodies (ANCA). The aim of this study was to determine the clinicopathologic predictors of patient and renal outcomes in anti-GBM disease with or without ANCA.

METHODS:

Retrospective review of 43 patients diagnosed with anti-GBM disease over 20 years in two centres, including nine with dual anti-GBM and ANCA positivity. Renal biopsies from 27 patients were scored for the presence of active and chronic lesions.

RESULTS:

Dual-positive patients were almost 20 years older than those with anti-GBM positivity alone (P = 0.003). The overall 1-year patient and renal survivals were 88 and 16%, respectively. Oligoanuria at diagnosis was the strongest predictor of mortality; none of the 16 patients without oligoanuria died. In a Cox regression model excluding oligoanuria, age was the only other independent predictor of survival. Pulmonary haemorrhage and dialysis dependence did not influence mortality. Thirty-five of the forty-three (81%) patients required dialysis at presentation, including all nine dual-positive patients. Of them, only two (5.7%) regained renal function at 1 year. By logistic regression, oligoanuria at diagnosis and percentage of crescents were independent predictors of dialysis independence at 3 months. However, in biopsied patients, the presence of crescents (>75%) added little to the presence of oligoanuria in predicting dialysis independence. Histological activity and chronicity indices did not predict renal outcome. Two of the nine (22%) dual-positive patients relapsed compared with none of the anti-GBM alone patients. Seven patients received kidney transplants without disease recurrence.

CONCLUSIONS:

Oligoanuria is the strongest predictor of patient and renal survival while percentage of glomerular crescents is the only pathologic parameter associated with poor renal outcome in anti-GBM disease. Kidney biopsy may not be necessary in oligoanuric patients without pulmonary haemorrhage.

• Authors: Bassam Alchi, Meryl Griffiths, Murugan Sivalingam, David Jayne, Ken Farrington
• Journal: Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association (Nephrol Dial Transplant) Vol. 30 Issue 5 Pg. 814-21 (May 2015) ISSN: 1460-2385 [Electronic] England
• PMID: 25609740 (Publication Type: Journal Article, Multicenter Study)
• Copyright: © The Author 2015. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Autoantibodies
• antiglomerular basement membrane antibody

Topics

• Adult
• Aged
• Aged, 80 and over
• Anti-Glomerular Basement Membrane Disease (diagnosis, immunology, mortality)
• Antibodies, Antineutrophil Cytoplasmic (immunology)
• Autoantibodies (immunology)
• Biopsy
• Female
• Hemorrhage (complications)
• Humans
• Kidney (physiopathology)
• Kidney Failure, Chronic (mortality)
• Kidney Glomerulus (immunology)
• Kidney Transplantation
• Lung Diseases (complications)
• Male
• Middle Aged
• Proportional Hazards Models
• Recurrence
• Renal Dialysis
• Retrospective Studies
• Treatment Outcome