Kidney nontumor
Anti-neutrophil cytoplasmic antibody (ANCA) associated renal disease
Granulomatosis with polyangiitis (Wegener's)


Topic Completed: 3 December 2012

Revised: 26 February 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Granulomatosis with polyangiitis [title] kidney
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Cite this page: Sangle N. Granulomatosis with polyangiitis (Wegener's). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneywegeners.html. Accessed March 18th, 2019.
Definition / general
  • Rare autoimmune disease of unknown etiology with triad of (a) necrotizing granulomatous inflammation of upper respiratory tract or lungs, (b) necrotizing pauci-immune vasculitis of small / medium sized vessels in lungs, upper airways and other sites; and (c) focal necrotizing glomerulonephritis (eMedicine: Granulomatosis with Polyangiitis [Accessed 9 January 2018])
  • “Limited” disease if only 1 or 2 of the above, usually spares kidneys
Terminology
Clinical features
  • Incidence of 3 per 100,000, occurs at any age but more common at ages 30 - 49 years
  • 80% have renal involvement, which progresses rapidly without treatment, leading to end stage renal disease (Scand J Rheumatol 2011;40:283) and death
  • Serum c-ANCA is 90% specific, 50% sensitive initially, 100% sensitive if active and generalized disease; c-ANCA not present during remission
Case reports
Treatment
  • Cyclophosphamide, corticosteroids (cause remission, but 50% relapse)
  • Renal transplant for end stage renal disease (Clin Transplant 2011;25:380)
Microscopic (histologic) description
  • Focal necrotizing glomerulonephritis, often with cellular crescents and usually with glomerular thrombosis (Hum Pathol 1984;15:943)
  • Interstitial inflammation common, renal papillary necrosis in 20%
  • Rare: granulomatous glomerulonephritis, necrotizing granulomas in biopsies
Microscopic (histologic) images

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Positive c-ANCA (immunofluorescence pattern produced by binding of ANCA to ethanol-fixed neutrophils)

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Various images

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Glomerulonephritis (left to right): focal necrotizing, sclerosing, early crescent


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Global cellular crescent

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Crescents (left to right): partial cellular, fibrin present (direct immunofluoresence with antibody panel)


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Vascular lesions (left to right): fibrinoid necrosis of interlobular artery; vasculitis

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Necrotizing granulomatous vasculitis

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Tubular disease: red blood cell casts

Immunofluorescence description
  • Usually no immune complex deposits, but fibrinogen in glomeruli and vessel walls, small amounts of IgM, IgG or C3 in glomeruli and vessels (pauci-immune)
Electron microscopy description
  • Rarely sparse electron dense deposits in glomeruli
Differential diagnosis
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