Kidney nontumor
Other primary glomerular diseases
Rapidly progressive (crescentic) glomerulonephritis TYPE 3

Author: Nikhil Sangle, M.D. (see Authors page)

Revised: 20 November 2018, last major update February 2014

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

Related topic: Rapidly progressive (crescentic) glomerulonephritis
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Cite this page: Sangle, N. Rapidly progressive (crescentic) glomerulonephritis TYPE 3. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneyrpgntype3.html. Accessed December 10th, 2019.
Definition / general
TYPE 3: Pauci-immune crescentic glomerulonephritis (50%)

  • No antiglomerular basement membrane antibodies, no immune complexes (hence called ‘pauci-immune’)
  • Usually elderly
  • Either limited to kidney or associated with clinical vasculitis (granulomatosis with polyangiitis (Wegener's), microscopic polyarteritis)
  • Rare complication of Sjogren's syndrome J Formos Med Assoc 2011;110:473)
  • Often have serum C-ANCA (against proteinase 3 PR3-ANCA; associated with granulomatosis with polyangiitis (Wegener's)) or P-ANCA (against myeloperoxidase MPO-ANCA; in those without extrarenal vasculitis)
  • Those with microscopic polyarteritis have either
  • Better prognosis than type 1
  • Poorer prognosis if high serum creatinine at presentation and focal C3c depositions in areas of glomerular and arteriolar fibrinoid necrosis (Int J Immunopathol Pharmacol 2012;25:287)
Microscopic (histologic) description
  • Resembles type 1
Microscopic (histologic) images

Images hosted on other servers:
Missing Image

Various images

Immunofluorescence description
  • Ig negative, possibly C3 and fibrin / fibrinogen with crescents (pauci-immune only refers to intensity of staining and does not imply that an immune process does not mediate the injury)
Electron microscopy images
  • Same as type 1 (no deposits, focal segmental glomerular necrosis and fibrin at glomerular basement membrane breaks)
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