Focal segmental necrotizing and crescentic lupus nephritis accompanied by perinuclear antineutrophil cytoplasmic antibody (P-ANCA) seropositivity is an unusual occurrence. We report the first biopsy-documented cases of membranous lupus nephritis class V with associated "pauci-immune" segmental necrotizing glomerulonephritis and P-ANCA seropositivity. The absence of subendothelial electron-dense deposits favored a manifestation of superimposed ANCA-associated glomerulonephritis rather than class III lupus nephritis. The pathogenetic implications of this association are explored.
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