title: Glomerular crescent formation in renal amyloidosis. A clinicopathological study and demonstration of upregulated cell-mediated immunity.
abstract: BACKGROUND
  Several studies examined glomerular crescents associated with renal amyloidosis. However, the incidence of crescents, the association between the 2 lesions, treatment and outcome are still controversial.
  
  
  METHODS
  We studied 107 consecutive biopsies of renal amyloidosis, and found cellular or fibrocellular crescents in 13 cases (12.1%). We investigated the clinical characteristics, pathological findings, treatment and outcome. We also performed immunohistochemical staining using T cell, macrophage and osteopontin (OPN) markers.
  
  
  RESULTS
  Amyloid was of the AA type in 12 cases, and all patients had rheumatoid arthritis. Six cases with AA amyloidosis had crescentic glomerulonephritis (CrGN), and 5 presented with rapidly progressive glomerulonephritis (RPGN). The percentage of crescents correlated negatively with serum albumin (r = -0.83, p < 0.001), and positively with serum creatinine (r = 0.72, p < 0.01) and urinary protein excretion (r = 0.85, p < 0.001). All RPGN patients developed end-stage renal disease, and 2 patients died shortly after treatment. Microscopic examination showed inflammatory cells within the glomeruli, and immunohistochemical study revealed abundant intrarenal T cells and macrophages in CrGN cases. Strong expression of OPN was observed in tubular epithelial cells and intraglomerular macrophages.
  
  
  CONCLUSIONS
  Cellular immune responses play a crucial role in glomerular crescents in renal amyloidosis. Immunosuppressive treatment is often ineffective and raises the risk of complications in CrGN with abundant glomerular sclerosis and tubulointerstitial injury.
authors.affiliation: Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kiyushu University, Fukuoka, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Masutani
authors.forename: M
authors.initials: M
authors.lastname: Nagata
authors.forename: H
authors.initials: H
authors.lastname: Ikeda
authors.forename: K
authors.initials: K
authors.lastname: Takeda
authors.forename: R
authors.initials: R
authors.lastname: Katafuchi
authors.forename: H
authors.initials: H
authors.lastname: Hirakata
authors.forename: K
authors.initials: K
authors.lastname: Tsuruya
authors.forename: M
authors.initials: M
authors.lastname: Iida
mesh_terms: D000328:Adult; D000368:Aged; D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications / Q000276:immunology / Q000473:pathology*; D015703:Antigens, CD / Q000276:immunology; D015214:Antigens, Differentiation, Myelomonocytic / Q000276:immunology; D001706:Biopsy; D017252:CD3 Complex / Q000276:immunology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005920:Glomerular Mesangium / Q000276:immunology / Q000378:metabolism / Q000648:ultrastructure; D005921:Glomerulonephritis / Q000209:etiology / Q000378:metabolism / Q000473:pathology; D006801:Humans; D007111:Immunity, Cellular; D007150:Immunohistochemistry; D007678:Kidney Glomerulus / Q000276:immunology / Q000378:metabolism / Q000648:ultrastructure*; D008264:Macrophages / Q000276:immunology / Q000473:pathology; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D053495:Osteopontin / Q000378:metabolism; D011379:Prognosis; D012189:Retrospective Studies; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology; D000097382:CD68 Molecule
chemical_list: D000682:Amyloid; D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D017252:CD3 Complex; D053495:Osteopontin; C067980:CD68 antigen, human; D000097382:CD68 Molecule