Skip to main content

Lund University Publications

LUND UNIVERSITY LIBRARIES

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

Hellmich, Bernhard ; Sanchez-Alamo, Beatriz ; Schirmer, Jan H. ; Berti, Alvise ; Blockmans, Daniel ; Cid, Maria C. ; Holle, Julia U. ; Hollinger, Nicole ; Karadag, Omer and Kronbichler, Andreas , et al. (2023) In Annals of the Rheumatic Diseases
Abstract

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... (More)

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.

(Less)
Please use this url to cite or link to this publication:
@article{53e86b3c-7530-4ef6-b6de-a17ea1b63b57,
  abstract     = {{<p>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.</p>}},
  author       = {{Hellmich, Bernhard and Sanchez-Alamo, Beatriz and Schirmer, Jan H. and Berti, Alvise and Blockmans, Daniel and Cid, Maria C. and Holle, Julia U. and Hollinger, Nicole and Karadag, Omer and Kronbichler, Andreas and Little, Mark A. and Luqmani, Raashid A. and Mahr, Alfred and Merkel, Peter A. and Mohammad, Aladdin J. and Monti, Sara and Mukhtyar, Chetan B. and Musial, Jacek and Price-Kuehne, Fiona and Segelmark, Mårten and Teng, Y. K.Onno and Terrier, Benjamin and Tomasson, Gunnar and Vaglio, Augusto and Vassilopoulos, Dimitrios and Verhoeven, Peter and Jayne, David}},
  issn         = {{0003-4967}},
  keywords     = {{cyclophosphamide; granulomatosis with polyangiitis; rituximab; systemic vasculitis}},
  language     = {{eng}},
  publisher    = {{BMJ Publishing Group}},
  series       = {{Annals of the Rheumatic Diseases}},
  title        = {{EULAR recommendations for the management of ANCA-associated vasculitis : 2022 update}},
  url          = {{http://dx.doi.org/10.1136/ard-2022-223764}},
  doi          = {{10.1136/ard-2022-223764}},
  year         = {{2023}},
}