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Nephrol Dial Transplant (1994) 9: 630-636
© 1994 European Renal Association-European Dialysis and Transplant Association


research-article

Does truly ‘idiopathic’ crescentic glomerulonephritis exist?

R. Angangco2, S. Thiru2, V. L. M. Esnault1, A. K. Short1, C. M. Lockwood1 and D. B. G. Oliveira1,

1Department of Medicine, University of Cambridge School of Clinical Medicine, Addenbrooke's Hospital Cambridge UK 2Department of Pathology, University of Cambridge School of Clinical Medicine, Addenbrooke's Hospital Cambridge UK

Correspondence and offprint requests to: Correspondence and offprint requests to: Dr D. B. G. Oliveira, Department of Medicine, Addenbrooke's Hospital, Hills Road, Cambridge CB22QQ UK

Crescentic glomerulonephritis is usually classified into antiglomerular basement membrane (GBM) disease, immune-complex disease, or pauci-immune crescentic nephritis. The last category includes patients with systemic vasculitis as well as ‘idiopathic’ isolated crescentic nephritis. The presence of anti-neutrophil cytoplasmic antibodies (ANCA) in many patients with apparently isolated crescentic nephritis suggests that this represents a renal-limited form of vasculitis, and that truly ‘idiopathic’ crescentic nephritis is a very rare entity.

We reviewed all renal biopsies with extracapillary proliferation seen at our centre since the availability of an ANCA assay (4-year period). There were 89 such biopsies of a total of 1240, of which 82 had sufficient details for further analysis. Of these, 10 had anti-GBM disease, 35 had epithelial proliferation associated with a variety of other diseases, and 36 had ANCA-associated disease. Nine of this last group had no extrarenal features and would previously have been classified as ‘idiopathic’ crescentic glomerulonephritis. The single remaining patient had an inactive glomerulonephritis with a scarred crescent; the predominant lesion was an interstitial nephritis.

We therefore conclude that truly ‘idiopathic’ crescentic nephritis is very rare, if it exists at all. The ability to provide a practically complete classification of crescentic nephritis has important prognostic and therapeutic consequences.

Keywords: ANCA; systemic vasculitis; autoimmunity; crescentic nephritis


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N. Hedger, J. Stevens, N. Drey, S. Walker, and P. Roderick
Incidence and outcome of pauci-immune rapidly progressive glomerulonephritis in Wessex, UK: a 10-year retrospective study
Nephrol. Dial. Transplant., October 1, 2000; 15(10): 1593 - 1599.
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