RecommendationEULAR recommendations for the management ofANCA-associated vasculitis:2022 updateAnn RheumBernhard Hellmich Beatriz Sanchez-Alamo,2 Jan H Schirmer,3 Alvise Berti5Dis:Daniel Blockmans,Maria C CidJulia U Holle,Nicole Hollinger,Omer Karadag,Andreas Kronbichler,10.11 Mark A Little,2 Raashid A Luqmani13 Alfred Mahr,4Peter A Merkel5 Aladdin J Mohammad,1116 Sara Monti7.18publisChetan B Mukhtyar Jacek Musial,2 Fiona Price-Kuehne,1 Marten Segelmark,21hedY K Onno Teng ,22 Benjamin Terrier ,3 Gunnar Tomasson 24.25Augusto Vaglio Dimitrios Vassilopoulos7 Peter Verhoeven,28David Jayne11a10.1136/ard-Handling editor Josef SABSTRACTIn 2009,the EULAR developed its first recom-SmolenBackground Since the publication of the EULARmendations for managing small and medium·2022Additional 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.These recommendations223only.To view,please visitin 2016,several randomised clinical trials have beenprovided guidance to clinicians and researchers andthe joumal online (http://dx.published that have the potential to change clinical carehave been widely cited.Recent landmark studies ondoi.org10.1136/ard-2022-223764.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 receptorMarend of article.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.2223Prof 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 AAV and areImmunologie,Medius Kliniken,biopsies andANCA testing to assist in establishingintended to give advice to clinicians,other healthAkademisches Lehrkran kenhausa diagnosis of AAV.For remission induction in life-professionals,pharmaceutical companies and regu-der Universitat Tubingen,Downloadedthreatening or organ-threatening AAV,we recommendatory organisations.Kirchheim unter Teck 73230,Germany;a combination of high-dose glucocorticoids(GCs)inb.hellmichmedius-kliniken.decombination with either rituximab or cyclophosphamide.METHODSomWe 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 procedure
