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NDT Advance Access originally published online on May 3, 2007
Nephrology Dialysis Transplantation 2007 22(7):2068-2071; doi:10.1093/ndt/gfm169
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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

Elastase-ANCA-associated idiopathic necrotizing crescentic glomerulonephritis—a report of three cases

Alexandre Seidowsky1, Maxime Hoffmann2, Sophie Ruben-Duval3, Rafik Mesbah1, Eric Masy4, Xavier Kyndt3, Béchir Maouad1, Stéphane Billion1, Laure Hélène Noël5, Philippe Vanhille3 and Pierre Bataille1

1Department of Nephrology, Boulogne sur Mer, Docteur Duchenne Hospital, 2Department of Nephrology, La Louvière Polyclinic, 3Department of Nephrology, Valenciennes Hospital, 4Immuno-Hématology Laboratory, Valenciennes Hospital and 5AP-HP, Necker Hospital, France

Correspondence and offprint requests to: Pierre Bataille, Department of Nephrology, Boulogne sur Mer, Docteur Duchenne Hospital, France. Email: ppierrebataille@aol.com

Keywords: acute renal failure; antineutrophil cytoplasmic antibodies; elastase; vasculitis

The first 150 words of the full text of this article appear below.



   Introduction
 
As it is well known, the two major antigens against anti-neutrophil cytoplasmic antibodies (ANCAs), proteinase 3 (PR3) and myeloperoxidase (MPO), are commonly observed in small-vessel systemic vasculitis. The diagnosis value of anti-PR3 antibody (PR3-ANCA) for Wegener's granulomatosis and anti-MPO antibody (MPO-ANCA) for idiopathic necrotizing crescentic glomerulonephritis (NCGN) and microscopic polyangiitis are well established. Human neutrophil elastase antibody (HNE-ANCA), a minor ANCA [1] is sometimes detected along with PR3-ANCA or MPO-ANCA in systemic vasculitis [2,3]. Although patients with drug-induced vasculitis or with cocaine-induced midline destructive lesions (CIMDL) without autoimmune vasculitis may be positive for only HNE-ANCA, the diagnostic value of only positive HNE-ANCA remains doubtful in microscopic polyangiitis and NCGN [4–6]. We report here three cases of NCGN associated with systemic vasculitis, which were positive for HNE-ANCA but not for MPO or PR3.



   Cases
 
Case 1
A 66-year-old male, smoker, was referred for asthenia, anorexia and . . . [Full Text of this Article]

Case 2
Case 3


   Discussion
 

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