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EULAR recommendations for the management of ANCA-associated vasculitis : 2022 update

EULAR recommendations for the management of ANCA-associated vasculitis : 2022 update

Title: EULAR recommendations for the management of ANCA-associated vasculitis : 2022 update
Author: Hellmich, Bernhard
Sanchez-Alamo, Beatriz
Schirmer, Jan H.
Berti, Alvise
Blockmans, Daniel
Cid, Maria C.
Holle, Julia U.
Hollinger, Nicole
Karadag, Omer
Kronbichler, Andreas
... 17 more authors Show all authors
Date: 2023-03-16
Language: English
Scope: 18
Department: Other departments
Faculty of Medicine
Series: Annals of the Rheumatic Diseases; 83(1)
ISSN: 0003-4967
DOI: 10.1136/ard-2022-223764
Subject: Gigtarlæknisfræði; cyclophosphamide; granulomatosis with polyangiitis; rituximab; systemic vasculitis; Cyclophosphamide/therapeutic use; Humans; Azathioprine/therapeutic use; Microscopic Polyangiitis/diagnosis; Remission Induction; Granulomatosis with Polyangiitis/diagnosis; Antibodies, Antineutrophil Cytoplasmic; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy; Rituximab/therapeutic use; Rheumatology; Immunology and Allergy; Immunology; General Biochemistry,Genetics and Molecular Biology
URI: https://hdl.handle.net/20.500.11815/4187

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Hellmich , B , Sanchez-Alamo , B , Schirmer , J H , Berti , A , Blockmans , D , Cid , M C , Holle , J U , Hollinger , N , Karadag , O , Kronbichler , A , Little , M A , Luqmani , R A , Mahr , A , Merkel , P A , Mohammad , A J , Monti , S , Mukhtyar , C B , Musial , J , Price-Kuehne , F , Segelmark , M , Teng , Y K O , Terrier , B , Tómasson , G , Vaglio , A , Vassilopoulos , D , Verhoeven , P & Jayne , D 2023 , ' EULAR recommendations for the management of ANCA-associated vasculitis : 2022 update ' , Annals of the Rheumatic Diseases , vol. 83 , no. 1 , 223764 . https://doi.org/10.1136/ard-2022-223764


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.


Funding Information: The authors wish to thank the librarian Oliver Weiner (Medical Department of the Kiel University Library, Kiel, Germany) for advice and assistance during the SLR. DJ was supported by the NIHR Cambridge Biomedical Research Centre. Publisher Copyright: © Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.

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