ANCA Glomerulonephritis and Vasculitis

Clin J Am Soc Nephrol. 2017 Oct 6;12(10):1680-1691. doi: 10.2215/CJN.02500317. Epub 2017 Aug 25.


ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). A diagnosis of ANCA vasculitis should always specify the serotype as MPO-ANCA positive, PR3-ANCA positive, or ANCA-negative. To fully characterize a patient, the serotype also should be accompanied by the clinicopathologic variant if this can be determined: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener), eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or renal-limited vasculitis. ANCA vasculitis is most prevalent in individuals >50 years old. There are racial/ethnic and geographic influences on the prevalence, serotype frequencies, and clinicopathologic phenotypes. There is clinical, in vitro, and animal model evidence that ANCAs cause disease by activating neutrophils to attack small vessels. Immunomodulatory and immunosuppressive therapies are used to induce remission, maintain remission, and treat relapses. Over recent years, there have been major advances in optimizing treatment by minimizing toxic therapy and utilizing more targeted therapy.

Keywords: ANCA; Animal; Antibodies; Antineutrophil Cytoplasmic; Autoantibodies; Autoimmunity; Churg-Strauss Syndrome; Granulomatosis with Polyangiitis; Microscopic Polyangiitis; Models; Myeloblastin; Neutrophil Activation; Neutrophils; Peroxidase; Prevalence; Recurrence; Serogroup; glomerulonephritis; vasculitis.

Publication types

  • Review

MeSH terms

  • Animals
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Churg-Strauss Syndrome* / diagnosis
  • Churg-Strauss Syndrome* / drug therapy
  • Churg-Strauss Syndrome* / epidemiology
  • Churg-Strauss Syndrome* / immunology
  • Female
  • Glomerulonephritis* / diagnosis
  • Glomerulonephritis* / drug therapy
  • Glomerulonephritis* / epidemiology
  • Glomerulonephritis* / immunology
  • Granulomatosis with Polyangiitis* / diagnosis
  • Granulomatosis with Polyangiitis* / drug therapy
  • Granulomatosis with Polyangiitis* / epidemiology
  • Granulomatosis with Polyangiitis* / immunology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Microscopic Polyangiitis* / diagnosis
  • Microscopic Polyangiitis* / drug therapy
  • Microscopic Polyangiitis* / epidemiology
  • Microscopic Polyangiitis* / immunology
  • Middle Aged
  • Myeloblastin / immunology
  • Peroxidase / immunology
  • Prevalence
  • Recurrence
  • Remission Induction
  • Risk Factors
  • Treatment Outcome


  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Peroxidase
  • Myeloblastin