ATLAS OF RENAL PATHOLOGY

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Pauci-immune Necrotizing Crescentic Glomerulonephritides: Wegener's Granulomatosis/Microscopic Polyangiitis

Pathology Editor: Agnes Fogo, MD
Medical Photographer: Brent Weedman
With Assistance From Kim Solez, MD, of the National Kidney Foundation's cyberNephrologyTM Team

 
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Fig 1. Segmental fibrinoid necrosis with nuclear debris and glomerular basement membrane disruption, typical of early stage of pauci-immune crescentic glomerulonephritis, diagnosed clinically as Wegener's granulomatosis in this case. The morphological differential includes microscopic polyangiitis and Wegener's granulomatosis, which cannot be distinguished morphologically from the kidney biopsy. Immunofluorescence studies are negative in both of these diseases, and few if any, electron dense areas are found by electron microscopy (Jones silver stain; original magnification x400).
 
 
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Fig 2. Well-developed cellular crescent with collapse of small amount of remaining glomerular tuft with segmental fibrinoid necrosis and extracapillary fibrin and necrosis in pauci-immune crescentic glomerulonephritis, consistent with Wegener's granulomatosis or microscopic polyangiitis. Immunofluorescence studies were negative (Jones silver stain; original magnification x400).
 
 
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Fig 3. Electron microscopy shows the absence of deposits along the capillary wall in a case of pauci-immune crescentic glomerulonephritis, consistent with Wegener's granulomatosis. The glomerular basement membrane break, which is the unit lesion leading to exudation of plasma proteins which stimulates proliferation of parietal epithelial cells, leading to crescent formation, is illustrated (transmission electron microscopy; original magnification x8,000).
 
 
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Fig 4. Fig 4. Open lung biopsy illustrating necrotizing granulomatous inflammation and vasculitis, with negative cultures typical and diagnostic of Wegener's granulomatosis (hematoxylin & eosin; original magnification x100).
 

From the Department of Pathology, Vanderbilt University Medical Center, Nashville, TN.
Address author queries to Agnes Fogo, MD, Department of Pathology, Vanderbilt University Medical Center, MCN C-3310, Nashville, TN 37232. E-mail:Agnes.Fogo@vanderbilt.edu
Am J Kidney Dis 32(2):E1, 1998 (available www.ajkd.org)
 Copyright 1998 by the National Kidney Foundation, Inc.

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