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Kidney non-tumor

Associated with systemic conditions

Wegener’s granulomatosis


Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 23 December 2012, last major update December 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● 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)
● “Limited” disease if only 1 or 2 of the above, usually spares kidneys

Clinical features
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● 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
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● 54 year old man with recovery of pulmonary involvement while renal disease progressed to end-stage.(Ren Fail 2011;33:1032)
● 73 year old man with fever, malaise, anorexia, 25 pound weight loss (University of Pittsburgh)

Treatment
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● Cyclophosphamide, corticosteroids (cause remission, but 50% relapse)
● Renal transplant for end stage renal disease (Clin Transplant 2011;25:380)

Micro description
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● 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

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


Various images

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


Global cellular crescent


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

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

   
Necrotizing granulomatous vasculitis


Tubular disease: red blood cell castss

Immunofluorescence
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● 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
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● Rarely sparse electron dense deposits in glomeruli

Differential diagnosis
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● Other pauci-immune glomerulonephritis, such as Churg-Strauss syndrome and microscopic polyangitis

End of Kidney non-tumor > Associated with systemic conditions > Wegener’s granulomatosis

Ref Updated: 8/10/12


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