Histopathological classification of antineutrophil cytoplasmic antibody-associated glomerulonephritis: an update

Curr Opin Nephrol Hypertens. 2014 May;23(3):224-31. doi: 10.1097/01.mnh.0000444818.95496.a4.


Purpose of review: This review discusses the findings of studies validating the histopathological classification of antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis, which was devised in 2010 by an international working group of pathologists and nephrologists in collaboration with the European Vasculitis Society.

Recent findings: So far, eight studies have validated the histopathological classification of ANCA-associated glomerulonephritis. The studies came from Japan, China, Australia, the United States, the Netherlands, and Turkey. These validation studies confirmed that the histopathological classification of ANCA-associated glomerulonephritis is of predictive value for renal outcome. This was especially the case for patients with either a focal or sclerotic-class renal biopsy, whereas the crescentic and mixed classes showed different results in the validation studies. These differences could be due to differences in patient populations or therapy, inter-rater reliability and lack of inclusion of tubulointerstitial lesions in the classification. Therapy is known to influence renal outcome, but due to the retrospective design of the to-date performed validation studies, this parameter could not be fully accounted for in these validation studies. Inter-rater reliability among three histopathologists was investigated in one study and was moderate.

Summary: The histopathological classification of ANCA-associated glomerulonephritis predicts renal outcome during follow-up, especially in patients with a focal or sclerotic-class renal biopsy. A large international validation study is currently being performed.

Video abstract: http://links.lww.com/CONH/A6.

Publication types

  • Review

MeSH terms

  • Animals
  • Antibodies, Antineutrophil Cytoplasmic / analysis*
  • Biomarkers / analysis
  • Biopsy
  • Glomerulonephritis / immunology
  • Glomerulonephritis / pathology*
  • Glomerulonephritis / therapy
  • Humans
  • Kidney Glomerulus / immunology
  • Kidney Glomerulus / pathology*
  • Predictive Value of Tests
  • Prognosis
  • Reproducibility of Results


  • Antibodies, Antineutrophil Cytoplasmic
  • Biomarkers