Azurocidin-specific-ANCA-related idiopathic necrotizing crescentic glomerulonephritis.
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.
Kimura
Rio
R
First Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan. rio@ac.catv.ne.jp
Matsuzawa
Naoki
N
Arimura
Yoshihiro
Y
Soejima
Akinori
A
Nakabayashi
Kimimasa
K
Yamada
Akira
A
D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D023181:Antimicrobial Cationic Peptides; D001798:Blood Proteins / Q000276:immunology*; D002352:Carrier Proteins / Q000276:immunology*; D005260:Female; D005921:Glomerulonephritis / Q000276:immunology* / Q000473:pathology; D006801:Humans
C062991:AZU1 protein, human; D019268:Antibodies, Antineutrophil Cytoplasmic; D023181:Antimicrobial Cationic Peptides; D001798:Blood Proteins; D002352:Carrier Proteins