Validation of a histopathologic classification scheme for antineutrophil cytoplasmic antibody-associated glomerulonephritis

Hum Pathol. 2014 Jul;45(7):1423-9. doi: 10.1016/j.humpath.2014.02.017. Epub 2014 Mar 6.


Antineutrophil cytoplasmic antibody-associated small-vessel vasculitides cause multiple organ system disease including rapidly progressive glomerulonephritis. Recently, Berden et al (J Am Soc Nephrol. 2010;21:1628-1636) proposed a new histopathologic classification scheme separating renal biopsies into 4 classes: focal, crescentic, mixed, and sclerotic. We validated the prognostic implications of this classification scheme in a retrospective cohort study of 67 individuals with antineutrophil cytoplasmic antibody glomerulonephritis who underwent kidney biopsy in Calgary, Alberta, between 2005 and 2010. Their biopsies were rescored according to the classification scheme of Berden et al. Additional tubulointerstitial parameters were also scored. Clinical information including demographics and creatinine values at presentation and 1-year follow-up was retrieved. The mean age was 60 years. Forty-one percent were female. Biopsies were classified as follows: 35% crescentic, 32% mixed, 21% focal, and 11% sclerotic. Ten patients (14%) died within 1 year. Among surviving patients, the overall mean (95% confidence interval) change in estimated glomerular filtration rate (eGFR) at 1 year was 11 (7-15) mL/min per 1.73 m(2), and this change significantly differed (P = .02) between the classes: 19 (11-27) mL/min per 1.73 m(2) with crescentic histology, 11 (1-21) mL/min per 1.73 m(2) with focal, 8 (3-13) mL/min per 1.73 m(2) with mixed, and -4 (-7 to -1) mL/min per 1.73 m(2) with sclerotic. Tubulointerstitial pathology parameters did not predict outcomes. Patients with crescentic class biopsies showed significantly more improvement in eGFR at 1 year compared with the mixed (P = .04) and sclerotic (P = .005) classes. The focal class was associated with the highest eGFR values at presentation and 1 year. These findings validate the prognostic utility of the Berden classification scheme and suggest that it may be generalizable.

Keywords: ANCA; Classification; Glomerulonephritis; Histology; Pathology; Prognosis; Vasculitis.

Publication types

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

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / pathology*
  • Antibodies, Antineutrophil Cytoplasmic / analysis
  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Female
  • Glomerulonephritis / classification*
  • Glomerulonephritis / immunology
  • Glomerulonephritis / pathology
  • Humans
  • Kidney / immunology
  • Kidney / pathology*
  • Male
  • Middle Aged
  • Prognosis


  • Antibodies, Antineutrophil Cytoplasmic