PMID- 34235880 OWN - NLM STAT- MEDLINE DCOM- 20210913 LR - 20210913 IS - 2151-4658 (Electronic) IS - 2151-464X (Linking) VI - 73 IP - 8 DP - 2021 Aug TI - 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. PG - 1088-1105 LID - 10.1002/acr.24634 [doi] AB - 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. CI - (c) 2021, American College of Rheumatology. FAU - Chung, Sharon A AU - Chung SA AD - University of California, San Francisco. FAU - Langford, Carol A AU - Langford CA AD - Cleveland Clinic, Cleveland, Ohio. FAU - Maz, Mehrdad AU - Maz M AUID- ORCID: 0000-0002-3594-0508 AD - University of Kansas Medical Center, Kansas City. FAU - Abril, Andy AU - Abril A AD - Mayo Clinic, Jacksonville, Florida. FAU - Gorelik, Mark AU - Gorelik M AD - Columbia University, New York, New York. FAU - Guyatt, Gordon AU - Guyatt G AD - McMaster University, Hamilton, Ontario, Canada. FAU - Archer, Amy M AU - Archer AM AD - Northwestern University, Chicago, Illinois. FAU - Conn, Doyt L AU - Conn DL AUID- ORCID: 0000-0003-3303-3944 AD - Emory University, Atlanta, Georgia. FAU - Full, Kathy A AU - Full KA AD - Springfield, Illinois. FAU - Grayson, Peter C AU - Grayson PC AUID- ORCID: 0000-0002-8269-9438 AD - National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, Maryland. FAU - Ibarra, Maria F AU - Ibarra MF AD - Children's Mercy Hospital, Kansas City, Missouri. FAU - Imundo, Lisa F AU - Imundo LF AD - Columbia University, New York, New York. FAU - Kim, Susan AU - Kim S AD - University of California, San Francisco. FAU - Merkel, Peter A AU - Merkel PA AUID- ORCID: 0000-0001-9284-7345 AD - University of Pennsylvania, Philadelphia. FAU - Rhee, Rennie L AU - Rhee RL AUID- ORCID: 0000-0002-4907-0304 AD - University of Pennsylvania, Philadelphia. FAU - Seo, Philip AU - Seo P AD - Johns Hopkins University, Baltimore, Maryland. FAU - Stone, John H AU - Stone JH AUID- ORCID: 0000-0001-6588-9435 AD - Massachusetts General Hospital, Boston. FAU - Sule, Sangeeta AU - Sule S AUID- ORCID: 0000-0002-5876-376X AD - Children's National Hospital, Washington, DC. FAU - Sundel, Robert P AU - Sundel RP AD - Boston Children's Hospital, Boston, Massachusetts. FAU - Vitobaldi, Omar I AU - Vitobaldi OI AD - Chicago, Illinois. FAU - Warner, Ann AU - Warner A AD - Saint Luke's Health System, Kansas City, Missouri. FAU - Byram, Kevin AU - Byram K AD - Vanderbilt University, Nashville, Tennessee. FAU - Dua, Anisha B AU - Dua AB AD - Northwestern University, Chicago, Illinois. FAU - Husainat, Nedaa AU - Husainat N AUID- ORCID: 0000-0002-9638-2992 AD - SSM Health-St. Mary's Hospital, St. Louis, Missouri. FAU - James, Karen E AU - James KE AD - University of Utah, Salt Lake City. FAU - Kalot, Mohamad A AU - Kalot MA AUID- ORCID: 0000-0002-6581-4561 AD - State University of New York at Buffalo. FAU - Lin, Yih Chang AU - Lin YC AD - University of South Florida, Tampa. FAU - Springer, Jason M AU - Springer JM AUID- ORCID: 0000-0002-3903-6049 AD - University of Kansas Medical Center, Kansas City. FAU - Turgunbaev, Marat AU - Turgunbaev M AD - American College of Rheumatology, Atlanta, Georgia. FAU - Villa-Forte, Alexandra AU - Villa-Forte A AD - Cleveland Clinic, Cleveland, Ohio. FAU - Turner, Amy S AU - Turner AS AUID- ORCID: 0000-0001-7695-2022 AD - American College of Rheumatology, Atlanta, Georgia. FAU - Mustafa, Reem A AU - Mustafa RA AUID- ORCID: 0000-0002-2091-0875 AD - University of Kansas Medical Center, Kansas City, and McMaster University, Hamilton, Ontario, Canada. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Systematic Review DEP - 20210708 PL - United States TA - Arthritis Care Res (Hoboken) JT - Arthritis care & research JID - 101518086 RN - 0 (Antibodies, Antineutrophil Cytoplasmic) RN - 0 (Biomarkers) RN - 0 (Immunosuppressive Agents) SB - IM MH - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis/*drug therapy/immunology MH - Antibodies, Antineutrophil Cytoplasmic/*blood MH - Biomarkers/blood MH - Clinical Decision-Making MH - Consensus MH - Decision Support Techniques MH - Evidence-Based Medicine/standards MH - Humans MH - Immunosuppressive Agents/adverse effects/*therapeutic use MH - Rheumatology/*standards MH - Severity of Illness Index MH - Treatment Outcome EDAT- 2021/07/09 06:00 MHDA- 2021/09/14 06:00 CRDT- 2021/07/08 07:13 PHST- 2020/09/25 00:00 [received] PHST- 2021/04/13 00:00 [accepted] PHST- 2021/07/09 06:00 [pubmed] PHST- 2021/09/14 06:00 [medline] PHST- 2021/07/08 07:13 [entrez] AID - 10.1002/acr.24634 [doi] PST - ppublish SO - Arthritis Care Res (Hoboken). 2021 Aug;73(8):1088-1105. doi: 10.1002/acr.24634. Epub 2021 Jul 8.