Azurocidin-specific-ANCA-related idiopathic necrotizing crescentic glomerulonephritis |
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Affiliation: | 1. Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy;2. Department of Surgical, Medical, Molecular and Critical Area Pathology, University of Pisa, Via Roma 67, Pisa 56126, Italy;3. Tor Vergata University, Rome, Italy;4. Department of Medicine and Surgery, University of Parma, Italy;5. Azienda Ospedaliero-Universitaria of Parma, Italy;6. Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy |
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Abstract: | An 80-year-old woman who had rapidly progressive glomerulonephritis unaccompanied by systemic vasculitis is described. On renal biopsy, she showed necrotizing crescentic glomerulonephritis by light microscopy and pauci-immune glomerular lesions by immunofluorescent study. No dense deposits were present on electronmicroscopic study. On serum examination, indirect immunofluorescent study showed perinuclear pattern antineutrophil cytoplasmic antibody (ANCA), but myeloperoxidase-ANCA and proteinase 3-ANCA were both negative. Her serum reacted only to azurocidin excluding other ANCA antigens: bactericidal permeability-increasing protein, cathepsin G, elastase, lactoferrin, or lysozyme. Serum creatinine level decreased, and C-reactive protein turned negative after steroid therapy. Azurocidin-ANCA also turned negative. It is suggested that azurocidin-ANCA might have been related to the inflammatory process of pauci-immune necrotizing crescentic glomerulonephritis in this patient. |
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Keywords: | Azurocidin antineutrophil cytoplasmic antibody (ANCA) necrotizing crescentic glomerulonephritis (NCGN) |
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