RecommendationEULAR recommendations for the management ofANCA-associated vasculitis:2022 updateBerhard HellmichBeatriz Sanchez-Alamoan H Schirmer,Alvise Berti5Daniel Blockmans,5 Maria C CidJulia U Holle,Nicole Hollinger,Omer Karadag,Andreas Kronbichler,0.11 Mark A Little,12 Raashid A Luqmani13 Alfred Mahr,4Peter A Merkel5 Aladdin J Mohammad1.16 Sara Monti17.18Chetan B Mukhtyar Jacek Musial,20 Fiona Price-Kuehne,11 Marten Segelmark,21Y K Onno Teng ,22 Benjamin Terrier,23 Gunnar Tomasson,2.25Augusto Vaglio Dimitrios Vassilopoulos7 Peter Verhoeven,2David Jayne1Handling editor Josef SABSTRACTIn 2009,the EULAR developed its first recom-SmolenBackground Since the publication of the EULARmendations for managing small and mediumAdditional supplementalrecommendations for the management of antineutrophilvessel vasculitis.3 An update focusing on AAVmaterial is published onlinecytoplasmic antibody (ANCA)-associated vasculitis (AAV)was published in 2016.5 These recommendationsonly.To view,please visitin 2016,several randomised clinical trials have beenprovided guidance to clinicians and researchers andthe journal online (http://dx.published that have the potential to change clinical carehave been widely cited.Recent landmark studies ondoi.org10.1136/ard-2022-2237640.and support the need for an update.the role of plasma exchange (PLEX),standardisa-Methods Using EULAR standardised operatingtion of glucocorticoid (GC)dosing,use of ritux-For numbered affiliations seeprocedures,the EULAR task force undertook aimab(RTX)for maintenance therapy,C5a receptorend of artice.systematic literature review and sought opinion from(C5aR)-targeted and anti-interleukin 5 (IL-5)20 experts from 16 countries.We modified existingtherapy in EGPA make this an opportune time toCorrespondence torecommendations and created new recommendations.update the 2016 guidelines.Prof Dr Bernhard Hellmich.Klinik fur Innere Medizin,Results Four overarching prindples and 17These recommendations address the diagnosisRheumatologie undrecommendations were formulated.We recommendand treatment of adult patients with AAvV and areImmunologie,Medius Kliniken,biopsies andANCA testing to assist in establishingintended to give advice to clinicians,other healthAkademisches Lehrkrankenhausa diagnosis of AAV.For remission induction in life-professionals,pharmaceutical companies and regu-der Universitat Tubingen,threatening or organ-threatening AAV,we recommendKirchheim unter Teck 73230,latory organisations.a combination of high-dose glucocorticoids(GCs)inb.hellmich medius-kliniken.decombination with either rituximab or cyclophosphamideMETHODSWe recommend tapering of the GC dose to a target ofThe recommendations were drafted according toReceived 13 December 20225mg prednisolone equivalent/day within 4-5months.Accepted 21 February 2023the 2014 update of the EULAR standardised oper-Avacopan may be considered as part of a strategy toating procedures for the development of EULAR-reduce exposure to GC in granulomatosis
