title: Glomerular capillary and endothelial cell injury is associated with the formation of necrotizing and crescentic lesions in crescentic glomerulonephritis.
abstract: BACKGROUND
  The associations of glomerular capillary and endothelial injury with the formation of necrotizing and crescentic lesions in cases of crescentic glomerulonephritis (GN) have not been evaluated in detail.
  
  
  METHODS
  Glomerular capillary and endothelial cell injury were assessed in renal biopsy specimens of crescentic GN, including those from patients with anti-neutrophil cytoplasmic autoantibodies (ANCA) -associated GN (n=45), anti-glomerular basement membrane (GBM) GN (n=7), lupus GN (n=21), and purpura GN (n=45) with light and electron microscopy and immunostaining for CD34.
  
  
  RESULTS
  In ANCA-associated GN, anti-GBM GN, lupus GN, and purpura GN, almost all active necrotizing glomerular lesions began as a loss of individual CD34-positive endothelial cells in glomerular capillaries, with or without leukocyte infiltration. Subsequently, necrotizing lesions developed and were characterized by an expansive loss of CD34-positive cells with fibrin exudation, GBM rupture, and cellular crescent formation. With electron microscopy, capillary destruction with fibrin exudation were evident in necrotizing and cellular crescentic lesions. During the progression to the chronic stage of crescentic GN, glomerular sclerosis developed with the disappearance of both CD34-positive glomerular capillaries and fibrocellular-to-fibrous crescents. In addition, the remaining glomerular lobes without crescents had marked collapsing tufts, a loss of endothelial cells, and the development of glomerular sclerosis.
  
  
  CONCLUSIONS
  The loss of glomerular capillaries with endothelial cell injury is commonly associated with the formation of necrotizing and cellular crescentic lesions, regardless of the pathogeneses associated with different types of crescentic GN, such as pauci-immune type ANCA-associated GN, anti-GBM GN, and immune-complex type GN. In addition, impaired capillary regeneration and a loss of endothelial cells contribute to the development of glomerular sclerosis with fibrous crescents and glomerular collapse.
authors: Department of Nephrology, Nippon Medical School.
authors: Emiko
authors: E
authors: Fujita
authors: Kiyotaka
authors: K
authors: Nagahama
authors: Akira
authors: A
authors: Shimizu
authors: Michiko
authors: M
authors: Aoki
authors: Seiichiro
authors: S
authors: Higo
authors: Fumihiko
authors: F
authors: Yasuda
authors: Akiko
authors: A
authors: Mii
authors: Megumi
authors: M
authors: Fukui
authors: Tomohiro
authors: T
authors: Kaneko
authors: Shuichi
authors: S
authors: Tsuruoka
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019867:Anti-Glomerular Basement Membrane Disease / Q000276:immunology / Q000473:pathology; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000276:immunology / Q000473:pathology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D018952:Antigens, CD34 / Q000032:analysis; D001323:Autoantibodies / Q000032:analysis; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D002196:Capillaries / Q000276:immunology / Q000473:pathology* / Q000648:ultrastructure; D002648:Child; D018450:Disease Progression; D042783:Endothelial Cells / Q000276:immunology / Q000473:pathology* / Q000648:ultrastructure; D005260:Female; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007678:Kidney Glomerulus / Q000098:blood supply*; D008181:Lupus Nephritis / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D016612:Paraffin Embedding; D016707:Tissue Fixation; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D018952:Antigens, CD34; D001323:Autoantibodies; D015415:Biomarkers; C051951:antiglomerular basement membrane antibody