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Practice Guideline
. 2021 Aug;73(8):1366-1383.
doi: 10.1002/art.41773. Epub 2021 Jul 8.

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Sharon A Chung  1 , Carol A Langford  2 , Mehrdad Maz  3 , Andy Abril  4 , Mark Gorelik  5 , Gordon Guyatt  6 , Amy M Archer  7 , Doyt L Conn  8 , Kathy A Full  9 , Peter C Grayson  10 , Maria F Ibarra  11 , Lisa F Imundo  5 , Susan Kim  1 , Peter A Merkel  12 , Rennie L Rhee  12 , Philip Seo  13 , John H Stone  14 , Sangeeta Sule  15 , Robert P Sundel  16 , Omar I Vitobaldi  17 , Ann Warner  18 , Kevin Byram  19 , Anisha B Dua  7 , Nedaa Husainat  20 , Karen E James  21 , Mohamad A Kalot  22 , Yih Chang Lin  23 , Jason M Springer  3 , Marat Turgunbaev  24 , Alexandra Villa-Forte  2 , Amy S Turner  24 , Reem A Mustafa  25
Affiliations
Practice Guideline

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis

Sharon A Chung et al. Arthritis Rheumatol. 2021 Aug.

Abstract

Objective: To provide evidence-based recommendations and expert guidance for the management of antineutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA).

Methods: Clinical questions regarding the treatment and management of AAV were developed in the population, intervention, comparator, and outcome (PICO) format (47 for GPA/MPA, 34 for EGPA). Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel.

Results: We present 26 recommendations and 5 ungraded position statements for GPA/MPA, and 15 recommendations and 5 ungraded position statements for EGPA. This guideline provides recommendations for remission induction and maintenance therapy as well as adjunctive treatment strategies in GPA, MPA, and EGPA. These recommendations include the use of rituximab for remission induction and maintenance in severe GPA and MPA and the use of mepolizumab in nonsevere EGPA. All recommendations are conditional due in part to the lack of multiple randomized controlled trials and/or low-quality evidence supporting the recommendations.

Conclusion: This guideline presents the first recommendations endorsed by the American College of Rheumatology and the Vasculitis Foundation for the management of AAV and provides guidance to health care professionals on how to treat these diseases.

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Conflict of interest statement

Financial Conflict: Forms submitted as required.

Figures

Figure 1.
Figure 1.
Key recommendations for the treatment of granulomatosis with polyangiitis and microscopic polyangiitis.
Figure 2.
Figure 2.
Key recommendations for the treatment of eosinophilic granulomatosis with polyangiitis.
See this image and copyright information in PMC

Comment in

References

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