Intended for healthcare professionals

Clinical Review State of the Art Review

Management of ANCA associated vasculitis

BMJ 2020; 368 doi: (Published 18 March 2020) Cite this as: BMJ 2020;368:m421
  1. Zachary S Wallace, assistant professor of medicine1 2 3,
  2. Eli M Miloslavsky, assistant professor of medicine2 3
  1. 1Clinical Epidemiology Program, Division of Rheumatology, Allergy, and Immunology, Mongan Institute, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA
  2. 2Rheumatology Unit, Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA
  3. 3Harvard Medical School, Boston, MA, USA
  1. Correspondence to: zswallace{at} (or @zach_wallace_md on Twitter)


Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a small to medium vessel vasculitis associated with excess morbidity and mortality. This review explores how management of AAV has evolved over the past two decades with pivotal randomized controlled trials shaping the management of induction and maintenance of remission. Contemporary AAV care is characterized by approaches that minimize the cumulative exposure to cyclophosphamide and glucocorticoids, increasingly use rituximab for remission induction and maintenance, and consider therapies with less toxicity (for example, methotrexate, mycophenolate mofetil) for manifestations of AAV that do not threaten organ function or survival. Simultaneously, improvements in outcomes, such as renal and overall survival, have been observed. Additional trials and observational studies evaluating the comparative effectiveness of agents for AAV in various patient subgroups are needed. Prospective studies are necessary to assess the effect of psychosocial interventions on patient reported outcomes in AAV. Despite the expanding array of treatments for AAV, little guidance on how to personalize AAV care is available to physicians.


  • Series explanation: State of the Art Reviews are commissioned on the basis of their relevance to academics and specialists in the US and internationally. For this reason they are written predominantly by US authors

  • Contributors: ZSW had the idea for the article. Both authors did the literature search and planned and wrote the article. ZSW accepts full responsibility for the finished article, had access to any data, and controlled the decision to publish; he is the guarantor.

  • Funding: ZSW receives funding from NIH/NIAMS (K23AR073334 and L30 AR070520).

  • Competing interests: We have read and understood the BMJ Group policy on declaration of interests and declare the follow interests: none.

  • Provenance and peer review: Commissioned; externally peer reviewed.

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