NDT Advance Access originally published online on January 8, 2007
Nephrology Dialysis Transplantation 2007 22(4):1020-1022; doi:10.1093/ndt/gfl776
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© The Author [2007]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org
Necrotizing crescentic glomerulonephritisa conditional knockout model discloses new therapeutic challenges*
Renal Immunopathology Laboratory, Fondazione DAmico per la Ricerca sulle Malattie Renali, Milan, Italy
Correspondence and offprint requests to: Maria Pia Rastaldi, Renal Immunopathology Laboratory, Fondazione DAmico per la Ricerca sulle Malattie Renali, c/o San Carlo Hospital, via Pio II, 3 20153 Milan, Italy. Email: mp.rastaldi@fastwebnet.it
Keywords: chemokine (C-X-C motif) receptor 4; rapidly progressive glomerulonephritis; von HippelLindau tumour suppressor protein
| The first 10% of the full text of this article appears below. |
Necrotizing extracapillary glomerulonephritis is considered the histological hallmark of pauci-immune renal vasculitis, a potentially life-threatening condition, in which glomerular involvement is mostly rapidly progressive [1]. A timely therapeutic intervention is mandatory to avoid the establishment of end-stage renal disease in a short period of time. First-line treatment consists of steroids and immunosuppressive drugs at high dosages that have radically improved the disease's prognosis, but that remain charged by frequent relapses, the appearance of side-effects, and do not obtain remission in 10% of the cases, with dramatic consequences for the patient [2].
Circulating anti-neutrophil cytoplasmic autoantibodies (ANCAs), present in about 90% of the cases, have