Renal pathology of ANCA-related vasculitis: proposal for standardization of pathological diagnosis in Japan

Clin Exp Nephrol. 2008 Aug;12(4):277-291. doi: 10.1007/s10157-008-0052-7. Epub 2008 Apr 22.


Background: In Japan, systematic evaluation of the histologic parameters of anti-neutrophil cytoplasmic autoantibodies (ANCA)-related vasculitis has been performed according to the Japanese classification by Shigematsu et al. However, this classification is quite different from that of the European Vasculitis Study Group (EUVAS) classification. Therefore, a histological common basis is needed to compare Japanese histological data with the international database.

Method: Histological parameters concerning glomerular, tubulointerstitial, and vascular lesions of ANCA-related vasculitis, which are indispensable for clinical management, were elucidated and defined by reviewing, utilizing the merits of, and amending the two scoring systems.

Results and conclusion: A new comprehensive and standardized scoring system, by which histological quantitative assessment can provide evidence for therapy planning, has been developed for renal biopsy of Japanese ANCA-related vasculitis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic* / immunology
  • Biopsy
  • Diagnostic Techniques and Procedures / standards*
  • Europe
  • Humans
  • Japan
  • Kidney / pathology*
  • Vasculitis / diagnosis*
  • Vasculitis / immunology
  • Vasculitis / pathology


  • Antibodies, Antineutrophil Cytoplasmic