Systematic review and meta-analysis for 2023 clinical practice guidelines of the Japan Research Committee of the Ministry of Health, Labour, and Welfare for Intractable Vasculitis for the management of ANCA-associated vasculitis

Mod Rheumatol. 2023 Aug 25;33(5):982-989. doi: 10.1093/mr/roac114.


Objectives: The objective of this study is to provide evidence for the revision of clinical practice guidelines for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis by the Japan Research Committee for Intractable Vasculitis.

Methods: PubMed, CENTRAL, and the Japan Medical Abstracts Society databases were searched for articles published between 2015 and 2020 to update the systematic review for existing clinical questions, while PubMed, CENTRAL, EMBASE, and the Japan Medical Abstracts Society were searched for articles published between 2000 and 2020 to conduct a systematic review for newly developed clinical questions. The certainty of evidence was assessed with the GRADE approach.

Results: For remission induction, when used in conjunction with cyclophosphamide or rituximab, reduced-dose glucocorticoid lowered the risk of serious adverse events compared to standard-dose glucocorticoid. Avacopan improved sustained remission at 12 months compared to high-dose glucocorticoid. Addition of plasma exchange to remission induction therapy did not reduce the risk of death, end-stage kidney disease, or relapse. For remission maintenance, rituximab reduced the risk of relapse compared to azathioprine. Long-term rituximab or azathioprine reduced the risk of relapse compared to short-term rituximab or azathioprine, respectively.

Conclusions: This systematic review provided evidence required to develop the 2023 clinical practice guideline for the management of ANCA-associated vasculitis.

Keywords: ANCA-associated vasculitis; GRADE system; granulomatosis with polyangiitis; microscopic polyangiitis; systematic review.

Publication types

  • Meta-Analysis
  • Systematic Review

MeSH terms

  • Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* / drug therapy
  • Antibodies, Antineutrophil Cytoplasmic
  • Azathioprine* / therapeutic use
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunosuppressive Agents
  • Japan
  • Recurrence
  • Remission Induction
  • Rituximab / therapeutic use


  • Azathioprine
  • Immunosuppressive Agents
  • Rituximab
  • Glucocorticoids
  • Antibodies, Antineutrophil Cytoplasmic