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2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis
Author(s) -
Chung Sharon A.,
Langford Carol A.,
Maz Mehrdad,
Abril Andy,
Gorelik Mark,
Guyatt Gordon,
Archer Amy M.,
Conn Doyt L.,
Full Kathy A.,
Grayson Peter C.,
Ibarra Maria F.,
Imundo Lisa F.,
Kim Susan,
Merkel Peter A.,
Rhee Rennie L.,
Seo Philip,
Stone John H.,
Sule Sangeeta,
Sundel Robert P.,
Vitobaldi Omar I.,
Warner Ann,
Byram Kevin,
Dua Anisha B.,
Husainat Nedaa,
James Karen E.,
Kalot Mohamad A.,
Lin Yih Chang,
Springer Jason M.,
Turgunbaev Marat,
VillaForte Alexandra,
Turner Amy S.,
Mustafa Reem A.
Publication year - 2021
Publication title -
arthritis care and research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.032
H-Index - 163
eISSN - 2151-4658
pISSN - 2151-464X
DOI - 10.1002/acr.24634
Subject(s) - medicine , granulomatosis with polyangiitis , microscopic polyangiitis , rituximab , guideline , vasculitis , eosinophilic , rheumatology , randomized controlled trial , population , anti neutrophil cytoplasmic antibody , intensive care medicine , physical therapy , pathology , disease , environmental health , lymphoma
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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