Abstract

Background The value of a histologic classification scheme to classify patients with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis (ANCA-GN) into focal, mixed, crescentic, and sclerotic types for predicting risk of end-stage renal disease (ESRD) is well documented. However, the prognostic value of histological classification specifically in elderly patients (≥70 years) with ANCA-GN has not previously been investigated. Methods Patients with biopsy-verified pauci-immune necrotizing glomerulonephritis were identified from the Norwegian Kidney Biopsy Registry between 1991 and 2012 and those ≥70 years of age at the time of diagnosis and having positive anti-neutrophil cytoplasmic antibody serology were included in this study. The incidence rate of ESRD and/or death was determined by linking the study cohort to the Norwegian Renal Registry and the Population Registry of Norway. The ESRD-free survival and patient survival were compared between the 4 histological types. Results Of the 81 patients included, 20 progressed to ESRD and 34 died. The 1-year and 5-year ESRD-free survival varied between histological groups (p = 0.003) as follows: focal, 97% and 97%, respectively; mixed, 70% and 57%; crescentic, 76% and 63%; and sclerotic, 49% and 49%. Patient survival did not differ significantly between groups (p = 0.30). Conclusion Histological classification in elderly patients with ANCA-GN is useful for predicting ESRD but not survival.

Highlights

  • Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), which affects multiple organs, has historically been associated with a high risk of long-term morbidity and mortality

  • Patients with biopsy-verified pauci-immune necrotizing glomerulonephritis were identified from the Norwegian Kidney Biopsy Registry between 1991 and 2012 and those ≥70 years of age at the time of diagnosis and having positive anti-neutrophil cytoplasmic antibody serology were included in this study

  • With regard to overall patient survival, Weiner et al reported a rate of 72% at 1 year and 65% at 2 years, compared to 72% and 64%, respectively, in our study cohort. This is the first study reporting the prognostic value of histological classification in elderly patients

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Summary

Introduction

Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV), which affects multiple organs, has historically been associated with a high risk of long-term morbidity and mortality. In the form of focal necrotizing glomerulonephritis (ANCA-GN), is frequent in patients with AAV and is associated with a poorer clinical outcome, including a substantial risk for end-stage renal disease (ESRD). The most frequently used and validated model to assess the prognostic information from kidney biopsies for ANCA-GN diagnosis is a histological classification scheme that classifies patients into 4 types: focal (≥50% normal glomeruli), crescentic (≥50% cellular crescents), mixed (

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