Pathogenesis of ANCA-Associated Pulmonary Vasculitis

Semin Respir Crit Care Med. 2018 Aug;39(4):413-424. doi: 10.1055/s-0038-1673386. Epub 2018 Nov 7.


Antineutrophil cytoplasmic antibodies (ANCAs) are autoantibodies specific for antigens located in the cytoplasmic granules of neutrophils and lysosomes of monocytes. ANCAs are associated with a spectrum of necrotizing vasculitis that includes granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. Pulmonary vasculitis and related extravascular inflammation and fibrosis are frequent components of ANCA vasculitis. In this review, we detail the factors that have been associated with the origin of the ANCA autoimmune response and summarize the most relevant clinical observations, in vitro evidence, and animal studies strongly indicating the pathogenic potential of ANCA. In addition, we describe the putative sequence of pathogenic mechanisms driven by ANCA-induced activation of neutrophils that result in small vessel necrotizing vasculitis and extravascular granulomatous necrotizing inflammation.

Publication types

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

MeSH terms

  • Animals
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / immunology*
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / physiopathology*
  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Disease Models, Animal
  • Humans
  • Lung / pathology*
  • Neutrophil Activation*
  • Neutrophils / immunology


  • Antibodies, Antineutrophil Cytoplasmic