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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., Competing Interests: Competing interests: AB received honoraria for consulting from GSK. AJM received speaker fees and/or consultancies from Amgen, Lilly, Vifor, Roche and GSK. AJM received speaker fees and/or consultancies from Amgen, Celgene, Chugai, Novartis, Roche and Vifor. AK received speaker fees and/or consultancies from Catalyst Biosciences, Walden Bioscience, Delta4, Otsuka, UriSalt and Vifor. BH received speaker fees and/or consultancies from AbbVie, Amgen, AstraZeneca, BMS, Boehringer, Chugai, GSK, InflaRx, Janssen, MSD, Pfizer, Novartis, Phadia, Roche and Vifor. BT received consulting fees from AstraZeneca, GlaxoSmithKline, Vifor and Pharma. DB received consultancies from Roche. DJ received speaker fees and/or consultancies from Amgen, AstraZeneca, BMS, Boehringer, Chemocentryx, Chugai, GSK, Novartis, Roche, Takeda and Vifor. DV received speaker fees and/or consultancies and/or grants from AbbVie, Genesis-Pharma, Janssen, Lilly, MSD, Novartis, Pfizer, Roche and UCB. The work is supported by the Greek Rheumatology Society and Professional Association of Rheumatologists (ERE-EPERE) and the Special Account for Research Grants (SARG), National and Kapodistrian University of Athens, Greece. JHS received research grants from the John Grube Foundation and the Deutsche Gesellschaft für Rheumatologie (German Society for Rheumatology). MAL received unrestricted research funding and consultancy fees from Vifor with Chemocentryx. MCC received consulting fees from GSK, Vifor, AbbVie and Janssen, and a research grant from Kiniksa Pharmaceuticals. MS has received consultancy fees from Hansa Biopharma, Vifor, AstraZeneca, Toleranzia and Chemocentryx. PM reports receiving funds for the following activities in the past 2 years: consulting: AbbVie, AstraZeneca, Boehringer-Ingelheim, Bristol-Myers Squibb, Chemocentryx, CSL Behring, Dynacure, EMDSerono, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, Immagene, InflaRx, Janssen, Kiniksa, Kyverna, Magenta, MiroBio, Mitsubishi, Neutrolis, Novartis, NS Pharma, Pfizer, Regeneron, Sparrow, Takeda and Talaris; research support: AbbVie, AstraZeneca, Boehringer-Ingelheim, Bristol-Myers Squibb, Chemocentryx, Eicos, Electra, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Sanofi and Takeda; stock options: Kyverna. OK reports receiving speaking fees and/or consultancies from Amgen, AbbVie, Lilly, UCB-Pharma, Novartis, Celltrion; and research support from AbbVie, Viela-Bio, Roche and Novartis. RAL received speaker fees and/or consultancies and/or grants from AbbVie, BMS/Celgene, Chemocentryx, Chugai, GSK, InflaRx, Pfizer Global, Roche and Vifor. The LUMC received on behalf of YKOT an unrestricted research grant from CSL Vifor, GlaxoSmithKline, Aurinia Pharmaceuticals. The LUMC received consulting fees from Aurinia Pharmaceuticals, Novartis, GSK, KezarBio, Vifor Pharma and Otsuka Pharmaceuticals on consultancies delivered by YKOT. The work of YKOT is supported by the Dutch Kidney Foundation (17OKG04) and by the Arthritis Research and Collaboration Hub (ARCH) foundation. ARCH is funded by Dutch Arthritis Foundation (ReumaNederland). AV, BS-A, CBM, FP-K, GT, JH, JM, NH, PV and SM reported no conflicts of interest., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)