Pathology of ANCA-associated vasculitis

Clin Exp Nephrol. 2013 Oct;17(5):652-658. doi: 10.1007/s10157-012-0701-8. Epub 2012 Nov 23.


According to the recent 2012 Chapel Hill Consensus Conference, ANCA-associated vasculitis is classified in the group of small vessel vasculitis. Pauci-immune necrotizing crescentic glomerulonephritis is the morphological hallmark of ANCA-associated vasculitis. Crescentic damage of the glomerular tuft is characterized by macrophage accumulation through vascular cell adhesion molecule-1 (VCAM-1) activation. Macrophages have a key role in chronic progression of renal damage due to production of substances involved in matrix remodelling [transforming growth factor-beta (TGFβ)]. Diffuse interstitial infiltration of T and B lymphocytes and macrophages is another frequent morphological feature. Tubulitis is considered an important marker of worse prognosis. Unfortunately, the renal changes on long-term follow-up are largely unknown due to the small number of studies with repeat biopsy in these disorders. Although a standardized score for renal biopsies was developed previously, a final histopathologic classification is still lacking. The European Vasculitis Study Group (EUVAS) proposed a classification system based on glomerular pathology as assessed by light microscopy.

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / complications
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / pathology*
  • Biomarkers / analysis
  • Biopsy
  • Disease Progression
  • Glomerulonephritis / immunology
  • Glomerulonephritis / pathology*
  • Humans
  • Immunohistochemistry
  • Kidney Glomerulus / immunology
  • Kidney Glomerulus / pathology*
  • Predictive Value of Tests
  • Prognosis
  • Severity of Illness Index


  • Biomarkers