Anti-neutrophil cytoplasmic antibodies and pathogenesis of small vessel vasculitides

Autoimmun Rev. 2003 May;2(3):158-64. doi: 10.1016/s1568-9972(03)00010-7.


Anti-neutrophil cytoplasmic antibodies (ANCA) are a heterogenous group of autoantibodies with a broad spectrum of clinically associated diseases. ANCA testing has been established as a useful tool for the diagnosis of small vessel vasculitides, especially of 'ANCA-associated vasculitides' (AAV), such as Wegener's granulomatosis, microscopic polyangiitis and Churg-Strauss syndrome, in which circulating ANCA are commonly found. Within the last 20 years these antibodies were subject of intensive studies and a growing body of evidence arose for a distinct role of ANCA in the pathogenesis of the AAV. Our current concept of whether ANCA directly or indirectly contribute to vascular damage (ANCA-cytokine-sequence-theory) was mainly developed from in vitro studies and is supported by data from clinical investigations as well as animal models. Recently a direct causal link between ANCA and the development of glomerulonephritis and vasculitis has been demonstrated. We now know that a passive transfer of ANCA is sufficient to induce disease, but it remains to be discovered how the autoantibodies to neutrophil antigens might triggered.

Publication types

  • Review

MeSH terms

  • Animals
  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Churg-Strauss Syndrome / diagnosis
  • Churg-Strauss Syndrome / immunology
  • Churg-Strauss Syndrome / pathology
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / immunology
  • Granulomatosis with Polyangiitis / pathology
  • Humans
  • Myeloblastin
  • Peroxidase / immunology
  • Serine Endopeptidases / immunology
  • Vasculitis / diagnosis
  • Vasculitis / etiology*
  • Vasculitis / immunology


  • Antibodies, Antineutrophil Cytoplasmic
  • Peroxidase
  • Serine Endopeptidases
  • Myeloblastin