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Table 3 Proposed diagnostic criteria for kidney involvement in AAV [16]

From: IgG4-related nephritis and interstitial pulmonary disease complicated by invasive pulmonary fungal infection: a case report

clinical manifestations a rapidly progressive GN with a decline in kidney function accompanied by sub–nephrotic-range proteinuria, microscopic hematuria, and hypertension over days to a few months
Serology Anti-MPO antibody or anti-PR3 antibody positive
Pathological findings pauci-immune focal necrotizing crescentic GN
Rare patients with AAV have a prominent tubulointerstitial nephritis, which can be associated with vasculitis of the vasa recta
  1. A diagnosis of AAV incorporates the integration of clinical features, ANCA serology, and tissue pathology as needed