Histopathological Classification-A Prognostic Tool for Rapidly Progressive Glomerulonephritis

Medicina (Kaunas). 2018 Apr 17;54(2):17. doi: 10.3390/medicina54020017.


Background: Recently proposed histopathological classification may predict patient outcome in pauci-immune glomerulonephritis. This study sought to prove that the prognostic effect could be extended to all types of rapidly progressive glomerulonephritis. Methods: Retrospective analysis of patients diagnosed with rapidly progressive glomerulonephritis between April 1999 and August 2015 was performed. Epidemiological and clinical data were collected from medical records. The descriptions of renal biopsies were reviewed and classified into focal, sclerotic, crescentic and mixed class according to classification proposed by Berden et al. The study end points were end stage renal disease (ESRD) or death. Survival analyses were modelled using Cox regression. Results: 73 renal biopsies with diagnosis of rapidly progressive glomerulonephritis were included in the study. 25 (34.2%), 16 (21.9%), 24 (32.9%) and 8 (11%) patients were assigned to focal, crescentic, mixed and sclerotic class, respectively. Thirty-two (42.5%) patients were anti-neutrophil cytoplasmic antibody (ANCA) negative, of which eight (10.9%) were anti⁻glomerular basement membrane antibody (anti⁻GBM) positive and 24 (32.8%) were negative for autoimmune antibodies. Six (8.2%) patients died within one year. Among patients who survived, median change in estimated glomerular filtration rate (eGFR) values were: -10.5 mL/min in focal, 4.2 mL/min in crescentic, -4.3 mL/min in mixed and 4.1 mL/min in sclerotic group, p > 0.05. In the Cox regression model, there was no significant predictor of patient survival whereas the sclerotic group (HR 3.679, 95% CI, 1.164⁻11.628, p < 0.05) and baseline eGFR of <15 mL/min (HR 4.832, 95% CI, 1.55⁻15.08, p < 0.01) had an unfavorable effect for renal survival. Conclusions: Predominant glomerular sclerosis and low eGFR at baseline are associated with higher risk of ESRD in cases with crescentic glomerulonephritis. Therefore, despite the origin of injury, histological classification might aid in prediction of patient outcomes in rapidly progressive glomerulonephritis.

Keywords: ANCA associated glomerulonephritis; crescentic glomerulonephritis; histopathological classification; rapidly progressive glomerulonephritis; renal outcome.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Azathioprine / therapeutic use
  • Biopsy
  • Cyclophosphamide / therapeutic use
  • Disease Progression
  • Female
  • Glomerulonephritis / classification
  • Glomerulonephritis / drug therapy
  • Glomerulonephritis / pathology*
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Induction Chemotherapy / methods
  • Kidney / pathology*
  • Maintenance Chemotherapy / methods
  • Male
  • Methotrexate / therapeutic use
  • Middle Aged
  • Prognosis
  • Retrospective Studies
  • Treatment Outcome


  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Azathioprine
  • Methotrexate