RT Journal Article
SR Electronic
T1 EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP ard-2022-223764
DO 10.1136/ard-2022-223764
A1 Bernhard Hellmich
A1 Beatriz Sanchez-Alamo
A1 Jan H Schirmer
A1 Alvise Berti
A1 Daniel Blockmans
A1 Maria C Cid
A1 Julia U Holle
A1 Nicole Hollinger
A1 Omer Karadag
A1 Andreas Kronbichler
A1 Mark A Little
A1 Raashid A Luqmani
A1 Alfred Mahr
A1 Peter A Merkel
A1 Aladdin J Mohammad
A1 Sara Monti
A1 Chetan B Mukhtyar
A1 Jacek Musial
A1 Fiona Price-Kuehne
A1 Mårten Segelmark
A1 Y K Onno Teng
A1 Benjamin Terrier
A1 Gunnar Tomasson
A1 Augusto Vaglio
A1 Dimitrios Vassilopoulos
A1 Peter Verhoeven
A1 David Jayne
YR 2023
UL http://ard.bmj.com/content/early/2023/03/16/ard-2022-223764.abstract
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.