Does truly 'idiopathic' crescentic glomerulonephritis exist?

Nephrol Dial Transplant. 1994;9(6):630-6. doi: 10.1093/ndt/9.6.630.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies / analysis*
  • Autoantibodies / blood
  • Biomarkers / analysis
  • Biopsy
  • Diagnosis, Differential
  • Female
  • Glomerulonephritis / classification
  • Glomerulonephritis / immunology
  • Glomerulonephritis / pathology*
  • Humans
  • Immune Complex Diseases / blood
  • Immune Complex Diseases / immunology
  • Immune Complex Diseases / pathology*
  • Kidney Glomerulus / blood supply
  • Kidney Glomerulus / immunology
  • Kidney Glomerulus / pathology*
  • Male
  • Middle Aged
  • Neutrophils / immunology
  • Vasculitis / immunology
  • Vasculitis / pathology

Substances

  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Biomarkers