Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
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Case Report

Volume 12, Number 1, January 2021, pages 27-31

Favorable Pregnancy Outcome in a Granulomatosis With Polyangiitis Patient With Renal Insufficiency


Table 1. Definitions for ANCA-Associated Vasculitis
ANCA: antineutrophil cytoplasmic antibodies; MPO: myeloperoxidase; PR3: proteinase 3.
ANCA-associated vasculitis (AAV)Necrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels, associated with MPO-ANCA or PR3-ANCA. Not all patients have ANCA. Add a prefix indicating ANCA reactivity, e.g., PR3-ANCA, MPO-ANCA, ANCA-negative.
Granulomatosis with polyangiitis (Wegener’s granulomatosis)Necrotizing granulomatous inflammation usually involving the upper and lower respiratory tract, and necrotizing vasculitis affecting predominantly small to medium vessels. Necrotizing glomerulonephritis is common.
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)Eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium vessels, and associated with asthma and eosinophilia. ANCA is more frequent when glomerulonephritis is present.
Microscopic polyangiitisNecrotizing vasculitis, with few or no immune deposits, predominantly affecting small vessels. Necrotizing arteritis involving small and medium arteries may be present. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis often occurs. Granulomatous inflammation is absent.


Table 2. Stages of Disease Based on Clinical and Pathologic Criteria
LimitedDisease localized to the upper airways, no systemic symptoms, no threatened organ function, no renal involvement
Early generalizedConstitutional symptoms, no threatened organ function
Active generalizedConstitutional symptoms with threatened organ function
SevereSevere renal involvement, life-threatening disease
RefractoryProgressive disease that is unresponsive to therapy