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 30
OP 47
DO 10.1136/ard-2022-223764
VO 83
IS 1
A1 Hellmich, Bernhard
A1 Sanchez-Alamo, Beatriz
A1 Schirmer, Jan H
A1 Berti, Alvise
A1 Blockmans, Daniel
A1 Cid, Maria C
A1 Holle, Julia U
A1 Hollinger, Nicole
A1 Karadag, Omer
A1 Kronbichler, Andreas
A1 Little, Mark A
A1 Luqmani, Raashid A
A1 Mahr, Alfred
A1 Merkel, Peter A
A1 Mohammad, Aladdin J
A1 Monti, Sara
A1 Mukhtyar, Chetan B
A1 Musial, Jacek
A1 Price-Kuehne, Fiona
A1 Segelmark, Mårten
A1 Teng, Y K Onno
A1 Terrier, Benjamin
A1 Tomasson, Gunnar
A1 Vaglio, Augusto
A1 Vassilopoulos, Dimitrios
A1 Verhoeven, Peter
A1 Jayne, David
YR 2024
UL http://ard.bmj.com/content/83/1/30.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.