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      Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis.

      American Journal of Kidney Diseases

      pathology, Aged, immunology, Vasculitis, Survival Analysis, Single-Blind Method, Serine Endopeptidases, Retrospective Studies, statistics & numerical data, Renal Replacement Therapy, Peroxidase, Neoplasm Proteins, Middle Aged, Microscopy, Fluorescence, Membrane Proteins, Male, Life Tables, blood supply, Kidney Glomerulus, etiology, epidemiology, Kidney Failure, Chronic, Kidney, Incidence, Humans, Granuloma, mortality, complications, Glomerulonephritis, Follow-Up Studies, Female, Disease Progression, blood, Creatinine, Biopsy, Basement Membrane, Autoimmune Diseases, Autoantigens, Antibody Specificity, Antibodies, Antineutrophil Cytoplasmic

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          In a substantial proportion of patients with crescentic glomerulonephritis (CGN), both anti-glomerular basement membrane (GBM) antibodies and antineutrophil cytoplasmic antibodies (ANCAs) with specificity for myeloperoxidase (MPO-ANCA) are detected. In the present study, we questioned whether histological and clinical features of patients with both ANCA and anti-GBM antibodies differ from those of patients with either ANCA or anti-GBM alone. We reviewed the Limburg renal biopsy registry (1978 to 2003; n = 1,373) for cases of CGN. The presence of linear fluorescence on renal biopsy and the presence of ANCA and/or anti-GBM antibodies were measured. Subsequently, we assessed patient characteristics and follow-up and compared histological findings among the different groups. We identified 46 MPO-ANCA-positive, 10 double-positive, and 13 anti-GBM-positive patients. Mean ages were 63, 64, and 52 years (P = 0.04), and serum creatinine levels were 5.0, 10.3, and 9.6 mg/dL (445, 910, and 850 micromol/L), respectively (P = 0.01). Granulomatous periglomerular inflammation was found in either MPO-ANCA- or double-positive patients, but not in anti-GBM-positive patients with CGN without MPO-ANCAs. Patient survival among the 3 groups was different, although not statistically significant (log rank P = 0.17, with 75%, 79%, and 100% alive at 1 year, respectively). Renal survival analysis showed significant differences among the 3 groups (P = 0.04, with 65%, 10%, and 15% off dialysis therapy at 1 year, respectively). In patients with both anti-GBM antibodies and MPO-ANCAs, histological findings differ from those of patients with anti-GBM antibodies only. However, renal survival in these patients is not better than that in anti-GBM-positive patients and is worse compared with patients with MPO-ANCAs only.

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