ANCA associated vasculitis (AAV): a review for internists

Postgrad Med. 2023 Jan;135(sup1):3-13. doi: 10.1080/00325481.2022.2102368. Epub 2022 Jul 21.


Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) compromise a rare group of necrotizing small to medium vessel vasculitides that constitute three distinct disorders: granulomatosis with polyangiitis (GPA) (formerly known as Wegener's granulomatosis), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) (formerly known as Churg-Strauss syndrome). AAV is characterized by the usual presence of circulating autoantibodies to the neutrophil proteins leukocyte proteinase 3 (PR3-ANCA) or myeloperoxidase (MPO-ANCA). These antibodies can activate neutrophils and the complement system resulting in vessel wall inflammation and damage. The clinical presentation of AAV varies from non-severe (non-life threatening) to severe often with potentially life-threatening multi-organ involvement. Early recognition and diagnosis are crucial. In the past two decades, advances in understanding the pathophysiology of AAV have led to development of new treatments and resulted in significant improvement in general outcomes and survival rates. This narrative review will focus on GPA and MPA. We will highlight clinical manifestations, diagnosis, disease monitoring, and treatment strategies in patients with AAV.

Keywords: ANCA associated vasculitis; advances in treatment; granulomatosis with polyangiitis (GPA); microscopic polyangiitis (MPA).

Publication types

  • Review

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / diagnosis
  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / therapy
  • Antibodies, Antineutrophil Cytoplasmic
  • Churg-Strauss Syndrome* / diagnosis
  • Churg-Strauss Syndrome* / therapy
  • Granulomatosis with Polyangiitis* / diagnosis
  • Granulomatosis with Polyangiitis* / therapy
  • Humans
  • Microscopic Polyangiitis*
  • Myeloblastin
  • Peroxidase


  • Antibodies, Antineutrophil Cytoplasmic
  • Myeloblastin
  • Peroxidase