PT  - JOURNAL ARTICLE
AU  - Bernhard Hellmich
AU  - Beatriz Sanchez-Alamo
AU  - Jan H Schirmer
AU  - Alvise Berti
AU  - Daniel Blockmans
AU  - Maria C Cid
AU  - Julia U Holle
AU  - Nicole Hollinger
AU  - Omer Karadag
AU  - Andreas Kronbichler
AU  - Mark A Little
AU  - Raashid A Luqmani
AU  - Alfred Mahr
AU  - Peter A Merkel
AU  - Aladdin J Mohammad
AU  - Sara Monti
AU  - Chetan B Mukhtyar
AU  - Jacek Musial
AU  - Fiona Price-Kuehne
AU  - Mårten Segelmark
AU  - Y K Onno Teng
AU  - Benjamin Terrier
AU  - Gunnar Tomasson
AU  - Augusto Vaglio
AU  - Dimitrios Vassilopoulos
AU  - Peter Verhoeven
AU  - David Jayne
TI  - EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update
AID  - 10.1136/ard-2022-223764
DP  - 2023 Mar 16
TA  - Annals of the Rheumatic Diseases
PG  - ard-2022-223764
4099  - http://ard.bmj.com/content/early/2023/03/16/ard-2022-223764.short
4100  - http://ard.bmj.com/content/early/2023/03/16/ard-2022-223764.full
AB  - 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.