EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update

Hellmich, Bernhard, Sanchez-Alamo, Beatriz, Schirmer, Jan H., Berti, Alvise, Blockmans, Daniel, Cid, Maria C., Holle, Julia U., Hollinger, Nicole, Karadag, Omer, Kronbichler, Andreas, Little, Mark A., Luqmani, Raashid A., Mahr, Alfred, Merkel, Peter A., Mohammad, Aladdin J., Monti, Sara, Mukhtyar, Chetan B. ORCID: https://orcid.org/0000-0002-9771-6667, Musial, Jacek, Price-Kuehne, Fiona, Segelmark, Mårten, Teng, Y. K. Onno, Terrier, Benjamin, Tomasson, Gunnar, Vaglio, Augusto, Vassilopoulos, Dimitrios, Verhoeven, Peter and Jayne, David (2024) EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Annals of the Rheumatic Diseases, 83 (1). pp. 30-47. ISSN 0003-4967

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Abstract

Background: Since the publication of the EULAR recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in 2016, several randomised clinical trials have been published that have the potential to change clinical care and support the need for an update. Methods: Using EULAR standardised operating procedures, the EULAR task force undertook a systematic literature review and sought opinion from 20 experts from 16 countries. We modified existing recommendations and created new recommendations. Results: Four overarching principles and 17 recommendations were formulated. We recommend biopsies and ANCA testing to assist in establishing a diagnosis of AAV. For remission induction in life-threatening or organ-threatening AAV, we recommend a combination of high-dose glucocorticoids (GCs) in combination with either rituximab or cyclophosphamide. We recommend tapering of the GC dose to a target of 5 mg prednisolone equivalent/day within 4-5 months. Avacopan may be considered as part of a strategy to reduce exposure to GC in granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). Plasma exchange may be considered in patients with rapidly progressive glomerulonephritis. For remission maintenance of GPA/MPA, we recommend rituximab. In patients with relapsing or refractory eosinophilic GPA, we recommend the use of mepolizumab. Azathioprine and methotrexate are alternatives to biologics for remission maintenance in AAV. Conclusions: In the light of recent advancements, these recommendations provide updated guidance on AAV management. As substantial data gaps still exist, informed decision-making between physicians and patients remains of key relevance.

Item Type: Article
Additional Information: Acknowledgements: The authors wish to thank the librarian Oliver Weiner (Medical Department of the Kiel University Library, Kiel, Germany) for advice and assistance during the SLR. DJ was supported by the NIHR Cambridge Biomedical Research Centre.
Uncontrolled Keywords: cyclophosphamide,granulomatosis with polyangiitis,rituximab,systemic vasculitis,rheumatology,immunology and allergy,immunology,biochemistry, genetics and molecular biology(all) ,/dk/atira/pure/subjectarea/asjc/2700/2745
Faculty \ School: Faculty of Medicine and Health Sciences > Norwich Medical School
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Depositing User: LivePure Connector
Date Deposited: 22 Oct 2023 01:09
Last Modified: 03 Jan 2024 03:17
URI: https://ueaeprints.uea.ac.uk/id/eprint/93392
DOI: 10.1136/ard-2022-223764

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