ANCA and associated diseases: immunodiagnostic and pathogenetic aspects

Clin Exp Immunol. 1993 Jan;91(1):1-12. doi: 10.1111/j.1365-2249.1993.tb03345.x.


The past decade has seen an explosion of data on the new group of autoantibodies known collectively as ANCA (anti-neutrophil cytoplasmic antibodies). ANCA are specific for granule proteins of granulocytes and monocytes and induce distinct fluorescence patterns, e.g. the cytoplasmic (classic) cANCA and the perinuclear pANCA. cANCA is induced by antibodies directed against Proteinase 3 (PR3; PR3-ANCA) in about 90% of all ANCA-positive sera, and pANCA is induced by antibodies against myeloperoxidase (MPO; MPO-ANCA) in about 40%. A further staining pattern, which does not have a clear cut association with a distinct granule protein, is sometimes seen in chronic inflammatory bowel diseases. PR3-ANCA are serological markers for Wegener's granulomatosis (WG) and MPO-ANCA are associated with certain subtypes of primary vasculitides. Evidence exists that both the autoantigen and ANCA participate in the pathogenesis of at least the group of 'ANCA-associated vasculitides'.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies / analysis*
  • Autoantigens / immunology
  • Chronic Disease
  • Endothelium / immunology
  • Granulomatosis with Polyangiitis / immunology*
  • Humans
  • Inflammatory Bowel Diseases / immunology
  • Kidney Diseases / immunology
  • Vasculitis / etiology
  • Vasculitis / immunology*


  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Autoantigens