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


METHODS
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.


RESULTS
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).


CONCLUSIONS
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.
Rutgers
Abraham
A
Department of Clinical and Experimental Immunology, Cardiovascular Research Institute Maastricht, Maastricht University, The Netherlands.
Slot
Marjan
M
van Paassen
Pieter
P
van Breda Vriesman
Peter
P
Heeringa
Peter
P
Tervaert
Jan Willem Cohen
JW
D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D000918:Antibody Specificity; D001324:Autoantigens / Q000276:immunology*; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology* / Q000401:mortality / Q000473:pathology; D001485:Basement Membrane / Q000276:immunology*; D001706:Biopsy; D003404:Creatinine / Q000097:blood; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000150:complications / Q000276:immunology* / Q000401:mortality / Q000473:pathology; D006099:Granuloma / Q000276:immunology / Q000473:pathology; D006801:Humans; D015994:Incidence; D007668:Kidney / Q000276:immunology / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000453:epidemiology / Q000209:etiology; D007678:Kidney Glomerulus / Q000098:blood supply; D015993:Life Tables; D008297:Male; D008565:Membrane Proteins / Q000276:immunology*; D008856:Microscopy, Fluorescence; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000276:immunology*; D009195:Peroxidase / Q000276:immunology*; D017582:Renal Replacement Therapy / Q000706:statistics & numerical data; D012189:Retrospective Studies; D012697:Serine Endopeptidases / Q000276:immunology*; D016037:Single-Blind Method; D016019:Survival Analysis; D014657:Vasculitis / Q000276:immunology / Q000473:pathology
D019268:Antibodies, Antineutrophil Cytoplasmic; D001324:Autoantigens; D008565:Membrane Proteins; D009363:Neoplasm Proteins; D003404:Creatinine; D009195:Peroxidase; D012697:Serine Endopeptidases; C409042:TMPRSS3 protein, human