호산구증다증을 동반한 P - A N CA 양성 괴사성 사구체신염

Abstract

Antineutrophil cytoplasmic antibodies (ANCAs) are now regarded as a serologic marker for pauci-immune crescentic necrotizing glomerulonephritis either in renal-limited form or in association with systemic vasculitis, such as Wegener’s granulomatosis, microscopic polyarteritis, and Churg-Strauss syndrome. Two major ANCA antigens have been indentified: proteinase3, which produces a cytoplasmic staining pattern termed C-ANCA, and myeloperoxidase, which produces a perinuclear pattern termed P-ANCA on ethanol-fixed neutrophils by indirect immunofluorescence. In ANCA- associated diseases, eosinphilia in excess of 1.5×1 09/L has been proposed to be characteristic of Churg-Strauss syndrome and is rare in other forms of ANCA-associated systemic vasculitis and crescentic necrotizing glomerulonephritis. Recently, there were two cases of P-ANCA positive crescentic necrotizing glomerulonephritis with peripheral blood eosinophilia and extrarenal microscopic vasculitis without asthma or granulomas. We experienced a patient with P-ANCA positive pauci-immune necrotizing glomerulonephritis with few eosinophilic infiltration and eosinophilia. He improved with oral prednisolone along with combination of intravenous cyclophosphamide. So we report this case with the review of literature.