Validation of the new classification of pauci-immune glomerulonephritis in a United States cohort and its correlation with renal outcome

BMC Nephrol. 2013 Oct 4;14:210. doi: 10.1186/1471-2369-14-210.


Background: Renal biopsies provide important diagnostic and prognostic information in ANCA associated glomerulonephritis. A new classification for prognostication of pauci-immune glomerulonephritis (GN) based on four categories (Mixed, Crescentic, Sclerotic and Focal) was proposed by an international working group of renal pathologists (IWGRP). The goal of our study was to apply the proposed classification system to a United States cohort of vasculitis patients and determine the association of IWGRP class with estimated glomerular filtration rate (eGFR) at one year.

Methods: Seventy-six cases of pauci-immune glomerulonephritis diagnosed from 1995 to 2011 from a single center were identified for this retrospective study. Clinical data were collected by abstraction from medical records. Histology was reviewed by a pathologist and classified according to the new classification. MDRD formula was used to calculate eGFR. We correlated IWGRP class to renal function at presentation and at one year. ×2, ANOVA, and linear regression analysis were performed as appropriate.

Results: Renal biopsies were categorized as focal: n = 20, crescentic: n = 18, mixed: n = 27, sclerotic: n = 11. The baseline e-GFR was lowest in the crescentic class and highest in the focal class. In linear regression analysis investigating e-GFR at 1 year; age and baseline e-GFR were independent predictors of e-GFR at 1 year.

Conclusions: The e-GFR at diagnosis and age were predictors of e-GFR at 1 year. Pathologic class at diagnosis may also be a helpful tool in risk stratification at diagnosis.

Publication types

  • Comparative Study
  • Validation Study

MeSH terms

  • Autoimmune Diseases / classification*
  • Autoimmune Diseases / pathology*
  • Autoimmune Diseases / physiopathology
  • Biopsy / methods
  • Cohort Studies
  • Female
  • Glomerular Filtration Rate*
  • Glomerulosclerosis, Focal Segmental / classification*
  • Glomerulosclerosis, Focal Segmental / pathology*
  • Glomerulosclerosis, Focal Segmental / physiopathology
  • Humans
  • Male
  • Middle Aged
  • Reproducibility of Results
  • Sensitivity and Specificity
  • Statistics as Topic
  • Terminology as Topic*
  • United States