Clinicopathological characteristics of typical and atypical anti-glomerular basement membrane nephritis

J Nephrol. 2017 Aug;30(4):503-509. doi: 10.1007/s40620-017-0394-x. Epub 2017 Apr 5.


Anti-glomerular basement membrane (GBM) antibody disease is a rare pathological condition that mainly involves renal and/or pulmonary parenchyma. It is characterized by the presence of circulating anti-GBM antibodies accompanied by a linear deposition of immunoglobulins (Ig) detected through immunofluorescence (IF) technique and typical signs and symptoms of organ dysfunction, such as rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage (PH). However, recently atypical forms of anti-GBM disease have been described and the presence of overlapping diseases contributed to make its diagnosis challenging. In this review will be discussed the entire spectrum of renal anti-GBM related conditions, focusing the attention on the differences in terms of pathogenesis, diagnosis and therapy of these disparate entities.

Keywords: Anti-GBM disease; Crescentic glomerulonephritis; Nephropathology; Rapidly progressive glomerulonephritis.

Publication types

  • Review

MeSH terms

  • Anti-Glomerular Basement Membrane Disease / classification
  • Anti-Glomerular Basement Membrane Disease / drug therapy
  • Anti-Glomerular Basement Membrane Disease / immunology
  • Anti-Glomerular Basement Membrane Disease / pathology*
  • Autoantibodies / immunology*
  • Biopsy
  • Drug Therapy, Combination
  • Fluorescent Antibody Technique
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Kidney Glomerulus / drug effects
  • Kidney Glomerulus / immunology
  • Kidney Glomerulus / pathology*
  • Kidney Glomerulus / ultrastructure
  • Microscopy, Electron
  • Predictive Value of Tests
  • Prognosis
  • Risk Factors


  • Autoantibodies
  • Immunosuppressive Agents
  • antiglomerular basement membrane antibody