Kidney non-tumor - Polyarteritis nodosa

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Kidney non-tumor

Associated with systemic conditions

Polyarteritis nodosa

Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 24 December 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Primary vasculitis of unknown etiology that affects muscular arteries at branch points
● Produces lesions of varying stages of evolution, also aneurysms
● Usually affects kidney (80%) and GI tract

Clinical features
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● 2 cases per million, 2/3 male, ages 50+ years
● 1/3 of cases are hepatitis B carriers
● Causes renal infarct, hypertension (often severe or malignant)
● May present with rapidly progressive renal insufficiency (Clin Nephrol 2010;74:315)
● < 20% have positive p-ANCA

Case reports
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● 36 year old man with bilateral renal hemorrhage wrongly attributed to blunt trauma (Nat Rev Urol 2009;6:563)
● 80 year old woman with MPO-ANCA (p-ANCA), fever, malaise and weight loss (Clin Exp Nephrol 2011;15:281)
● Case limited to kidneys (Hum Pathol 1987;18:1074)

Gross images
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Fatal retroperitoneal hemorrhage (red arrow), pseudoaneurysms (black arrows), multiple pale infarcts (white arrows)

Micro description
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● Necrotizing vasculitis of renal, interlobar and arcuate arteries at branch points with aneurysmal dilatation, fibrinoid necrosis and neutrophilic infiltration of vessel wall, often with thrombosis
● Later have mononuclear infiltrate, fibrosis of media and perivascular tissue and recanalization of thrombosed vessel
● Often not detected in biopsy since lesions are focal
● Glomeruli show ischemic changes of collapse and sclerosis

Micro images
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Lymphocytic vasculitis of renal vessels

Neutrophilic infiltration of vessels

Inflammation and fibrinoid necrosis

Small artery has inflammation and red staining (fibrinoid) due to penetration of plasma into vessel wall

Zone of infarction with coagulative necrosis on left, acute inflammation in middle, congested cortical parenchyma on right

Immunofluorescence
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● No deposits

Immunofluorescence images
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Irregular circumferential transmural staining for fibrin

Electron microscopy description
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● No deposits

Differential diagnosis
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● Kawasaki's disease: more mural edema and infiltrating monocytes, less fibrinoid necrosis

End of Kidney non-tumor > Associated with systemic conditions > Polyarteritis nodosa

Ref Updated: 8/7/12

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