. 2024 Jan 2;83(1):30-47.

doi: 10.1136/ard-2022-223764.

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

Bernhard Hellmich¹, Beatriz Sanchez-Alamo², Jan H Schirmer³, Alvise Berti^{4

5}, Daniel Blockmans⁶, Maria C Cid⁷, Julia U Holle⁸, Nicole Hollinger⁹, Omer Karadag¹⁰, Andreas Kronbichler^{11

12}, Mark A Little¹³, Raashid A Luqmani¹⁴, Alfred Mahr¹⁵, Peter A Merkel¹⁶, Aladdin J Mohammad^{12

17}, Sara Monti^{18

19}, Chetan B Mukhtyar²⁰, Jacek Musial²¹, Fiona Price-Kuehne¹², Mårten Segelmark²², Y K Onno Teng²³, Benjamin Terrier²⁴, Gunnar Tomasson^{25

26}, Augusto Vaglio²⁷, Dimitrios Vassilopoulos²⁸, Peter Verhoeven²⁹, David Jayne¹²

Affiliations

¹ Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany b.hellmich@medius-kliniken.de.
² Nephrology Department, Hospital Universitario del Sureste, Arganda del Rey, Spain.
³ Rheumatology & Clinical Immunology and Cluster of Excellence Precision Medicine in Chronic Inflammation, University Medical Center Schleswig-Holstein, Campus Kiel, Kiel, Germany.
⁴ CIBIO, Universita degli Studi di Trento, Trento, Italy.
⁵ Rheumatology, Santa Chiara Hospital, Trento, Italy.
⁶ Department of Internal Medicine, University Hospital of Leuven, Leuven, Belgium.
⁷ Department of Autoimmune Diseases, Hospital Clinic, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain.
⁸ Rheumazentrum Schleswig-Holstein Mitte, Neumuenster, Germany.
⁹ Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany.
¹⁰ Division of Rheumatology, Department of Internal Medicine, Vasculitis Research Center, Hacettepe University School of Medicine, Anakra, Turkey.
¹¹ Department of Internal Medicine IV, Medical University, Innsbruck, Austria.
¹² Department of Medicine, University of Cambridge, Cambridge, UK.
¹³ Trinity Health Kidney Centre, Trinity College Dublin, Dublin, Ireland.
¹⁴ Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science (NDORMs), University of Oxford, Oxford, UK.
¹⁵ Klinik für Rheumatologie, Kantonspital St Gallen, St Gallen, Switzerland.
¹⁶ Division of Rheumatology, Department of Medicine, Division of Epidemiology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
¹⁷ Department of Clinical Sciences, Lund University & Department of Rheumatology, Skåne Hospital, Lund, Sweden.
¹⁸ Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy.
¹⁹ Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
²⁰ Vasculitis Service, Rheumatology Department, Norfolk and Norwich University Hospital NHS Trust, Norwich, UK.
²¹ 2nd Department of Internal Medicine, Jagiellonian University Medical College, Kraków, Poland.
²² Division of Nephrology, Department of Clinical Sciences, Lund University, Skane University Hospital, Lund, Sweden.
²³ Centre of Expertise for Lupus-, Vasculitis-, and Complement-Mediated Systemic Autoimmune Diseases (LuVaCs), Department of Internal Medicine, Section Nephrology, Leiden University Medical Centre, Leiden, The Netherlands.
²⁴ National Referral Center for Rare Systemic Autoimmune Diseases, Université Paris Descartes, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.
²⁵ Department of Epidemiology and Biostatistics, Faculty of Medicine, University of Iceland, Reykjavik, Iceland.
²⁶ Department of Rheumatology and Centre for Rheumatology Research, University Hospital Reykjavik, Reykjavik, Iceland.
²⁷ Nephrology Unit, Meyer Children's Hospital, and Department of Biomedical, Experimental and Clinical Science, University of Florence, Florence, Italy.
²⁸ 2nd Department of Medicine and Laboratory, Clinical Immunology-Rheumatology Unit, National and Kapodistrian University of Athens, School of Medicine, Hippokration General Hospital, Athens, Greece.
²⁹ Dutch Patient Vasculitis Organization, Haarlem, The Netherlands.

PMID: 36927642
DOI: 10.1136/ard-2022-223764

Free article

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

Bernhard Hellmich et al. Ann Rheum Dis. 2024.

Free article

. 2024 Jan 2;83(1):30-47.

doi: 10.1136/ard-2022-223764.

Authors

Affiliations

¹ Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany b.hellmich@medius-kliniken.de.
² Nephrology Department, Hospital Universitario del Sureste, Arganda del Rey, Spain.
³ Rheumatology & Clinical Immunology and Cluster of Excellence Precision Medicine in Chronic Inflammation, University Medical Center Schleswig-Holstein, Campus Kiel, Kiel, Germany.
⁴ CIBIO, Universita degli Studi di Trento, Trento, Italy.
⁵ Rheumatology, Santa Chiara Hospital, Trento, Italy.
⁶ Department of Internal Medicine, University Hospital of Leuven, Leuven, Belgium.
⁷ Department of Autoimmune Diseases, Hospital Clinic, University of Barcelona, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain.
⁸ Rheumazentrum Schleswig-Holstein Mitte, Neumuenster, Germany.
⁹ Klinik für Innere Medizin, Rheumatologie und Immunologie, Medius Kliniken, Akademisches Lehrkrankenhaus der Universität Tübingen, Kirchheim unter Teck, Germany.
¹⁰ Division of Rheumatology, Department of Internal Medicine, Vasculitis Research Center, Hacettepe University School of Medicine, Anakra, Turkey.
¹¹ Department of Internal Medicine IV, Medical University, Innsbruck, Austria.
¹² Department of Medicine, University of Cambridge, Cambridge, UK.
¹³ Trinity Health Kidney Centre, Trinity College Dublin, Dublin, Ireland.
¹⁴ Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science (NDORMs), University of Oxford, Oxford, UK.
¹⁵ Klinik für Rheumatologie, Kantonspital St Gallen, St Gallen, Switzerland.
¹⁶ Division of Rheumatology, Department of Medicine, Division of Epidemiology, Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
¹⁷ Department of Clinical Sciences, Lund University & Department of Rheumatology, Skåne Hospital, Lund, Sweden.
¹⁸ Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy.
¹⁹ Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
²⁰ Vasculitis Service, Rheumatology Department, Norfolk and Norwich University Hospital NHS Trust, Norwich, UK.
²¹ 2nd Department of Internal Medicine, Jagiellonian University Medical College, Kraków, Poland.
²² Division of Nephrology, Department of Clinical Sciences, Lund University, Skane University Hospital, Lund, Sweden.
²³ Centre of Expertise for Lupus-, Vasculitis-, and Complement-Mediated Systemic Autoimmune Diseases (LuVaCs), Department of Internal Medicine, Section Nephrology, Leiden University Medical Centre, Leiden, The Netherlands.
²⁴ National Referral Center for Rare Systemic Autoimmune Diseases, Université Paris Descartes, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.
²⁵ Department of Epidemiology and Biostatistics, Faculty of Medicine, University of Iceland, Reykjavik, Iceland.
²⁶ Department of Rheumatology and Centre for Rheumatology Research, University Hospital Reykjavik, Reykjavik, Iceland.
²⁷ Nephrology Unit, Meyer Children's Hospital, and Department of Biomedical, Experimental and Clinical Science, University of Florence, Florence, Italy.
²⁸ 2nd Department of Medicine and Laboratory, Clinical Immunology-Rheumatology Unit, National and Kapodistrian University of Athens, School of Medicine, Hippokration General Hospital, Athens, Greece.
²⁹ Dutch Patient Vasculitis Organization, Haarlem, The Netherlands.

PMID: 36927642
DOI: 10.1136/ard-2022-223764

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 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.

Keywords: cyclophosphamide; granulomatosis with polyangiitis; rituximab; systemic vasculitis.

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Conflict of interest statement

Competing interests: AB received honoraria for consulting from GSK. AJM received speaker fees and/or consultancies from Amgen, Lilly, Vifor, Roche and GSK. AJM received speaker fees and/or consultancies from Amgen, Celgene, Chugai, Novartis, Roche and Vifor. AK received speaker fees and/or consultancies from Catalyst Biosciences, Walden Bioscience, Delta4, Otsuka, UriSalt and Vifor. BH received speaker fees and/or consultancies from AbbVie, Amgen, AstraZeneca, BMS, Boehringer, Chugai, GSK, InflaRx, Janssen, MSD, Pfizer, Novartis, Phadia, Roche and Vifor. BT received consulting fees from AstraZeneca, GlaxoSmithKline, Vifor and Pharma. DB received consultancies from Roche. DJ received speaker fees and/or consultancies from Amgen, AstraZeneca, BMS, Boehringer, Chemocentryx, Chugai, GSK, Novartis, Roche, Takeda and Vifor. DV received speaker fees and/or consultancies and/or grants from AbbVie, Genesis-Pharma, Janssen, Lilly, MSD, Novartis, Pfizer, Roche and UCB. The work is supported by the Greek Rheumatology Society and Professional Association of Rheumatologists (ERE-EPERE) and the Special Account for Research Grants (SARG), National and Kapodistrian University of Athens, Greece. JHS received research grants from the John Grube Foundation and the Deutsche Gesellschaft für Rheumatologie (German Society for Rheumatology). MAL received unrestricted research funding and consultancy fees from Vifor with Chemocentryx. MCC received consulting fees from GSK, Vifor, AbbVie and Janssen, and a research grant from Kiniksa Pharmaceuticals. MS has received consultancy fees from Hansa Biopharma, Vifor, AstraZeneca, Toleranzia and Chemocentryx. PM reports receiving funds for the following activities in the past 2 years: consulting: AbbVie, AstraZeneca, Boehringer-Ingelheim, Bristol-Myers Squibb, Chemocentryx, CSL Behring, Dynacure, EMDSerono, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, Immagene, InflaRx, Janssen, Kiniksa, Kyverna, Magenta, MiroBio, Mitsubishi, Neutrolis, Novartis, NS Pharma, Pfizer, Regeneron, Sparrow, Takeda and Talaris; research support: AbbVie, AstraZeneca, Boehringer-Ingelheim, Bristol-Myers Squibb, Chemocentryx, Eicos, Electra, Forbius, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Sanofi and Takeda; stock options: Kyverna. OK reports receiving speaking fees and/or consultancies from Amgen, AbbVie, Lilly, UCB-Pharma, Novartis, Celltrion; and research support from AbbVie, Viela-Bio, Roche and Novartis. RAL received speaker fees and/or consultancies and/or grants from AbbVie, BMS/Celgene, Chemocentryx, Chugai, GSK, InflaRx, Pfizer Global, Roche and Vifor. The LUMC received on behalf of YKOT an unrestricted research grant from CSL Vifor, GlaxoSmithKline, Aurinia Pharmaceuticals. The LUMC received consulting fees from Aurinia Pharmaceuticals, Novartis, GSK, KezarBio, Vifor Pharma and Otsuka Pharmaceuticals on consultancies delivered by YKOT. The work of YKOT is supported by the Dutch Kidney Foundation (17OKG04) and by the Arthritis Research and Collaboration Hub (ARCH) foundation. ARCH is funded by Dutch Arthritis Foundation (ReumaNederland). AV, BS-A, CBM, FP-K, GT, JH, JM, NH, PV and SM reported no conflicts of interest.

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Romero Gómez C, Ayala Gutiérrez MDM, Hernández Negrín H. Romero Gómez C, et al. Med Clin (Barc). 2024 Feb 9;162(3):141. doi: 10.1016/j.medcli.2023.07.025. Epub 2023 Nov 30. Med Clin (Barc). 2024. PMID: 38040572 English, Spanish. No abstract available.

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

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

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