{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T10:40:04Z","timestamp":1785580804148,"version":"3.56.0"},"reference-count":123,"publisher":"BMJ","issue":"3","license":[{"start":{"date-parts":[[2023,7,21]],"date-time":"2023-07-21T00:00:00Z","timestamp":1689897600000},"content-version":"unspecified","delay-in-days":20,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100008741","name":"EULAR","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100008741","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2023,5,16]]},"published-print":{"date-parts":[[2023,7]]},"abstract":"<jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>To summarise and update evidence to inform the 2022 update of the EULAR recommendations for the management of antineutrophil cytoplasm antibody-associated vasculitis (AAV).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>A systematic literature review (SLR) was performed to identify current evidence regarding treatment of AAV. PubMed, EMBASE and the Cochrane library were searched from 1 February 2015 to 25 February 2022. The evidence presented here is focused on the treatment of granulomatosis with polyangiitis and microscopic polyangiitis.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>3517 articles were screened and 175 assessed by full-text review. Ninety articles were included in the final evidence synthesis. Cyclophosphamide and rituximab (RTX) show similar efficacy for remission induction (level of evidence (LoE) 1a) but RTX is more effective in relapsing disease (LoE 1b). Glucocorticoid (GC) protocols with faster tapering result in similar remission rates but lower rates of serious infections (LoE 1b). Avacopan can be used to rapidly taper and replace GC (LoE 1b). Data on plasma exchange are inconsistent depending on the analysed trial populations but meta-analyses based on randomised controlled trials demonstrate a reduction of the risk of end-stage kidney disease at 1\u2009year but not during long-term follow-up (LoE 1a). Use of RTX for maintenance of remission is associated with lower relapse rates compared with azathioprine (AZA, LoE 1b). Prolonged maintenance treatment results in lower relapse rates for both, AZA (LoE 1b) and RTX (LoE 1b).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>This SLR provides current evidence to inform the 2022 update of the EULAR recommendations for the management of AAV.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/rmdopen-2023-003082","type":"journal-article","created":{"date-parts":[[2023,7,21]],"date-time":"2023-07-21T13:27:35Z","timestamp":1689946055000},"page":"e003082","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":26,"title":["Systematic literature review informing the 2022 update of the EULAR recommendations for the management of ANCA-associated vasculitis (AAV): part 1\u2014treatment of granulomatosis with polyangiitis and microscopic polyangiitis"],"prefix":"10.1136","volume":"9","clinical-trial-number":[{"clinical-trial-number":"nct03942887","registry":"10.18810\/clinical-trials-gov"}],"author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5467-2783","authenticated-orcid":false,"given":"Jan Henrik","family":"Schirmer","sequence":"first","affiliation":[{"name":"Clinic for Internal Medicine I, Rheumatology and Clinical Immunology, University Medical Center Schleswig-Holstein Campus Kiel, Kiel, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7835-543X","authenticated-orcid":false,"given":"Beatriz","family":"Sanchez-Alamo","sequence":"additional","affiliation":[{"name":"Nephrology, Hospital Universitario del Sureste, Arganda del Rey, Madrid, Spain"},{"name":"Nephrology, Sk\u00e5ne University Hospital, Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8014-1801","authenticated-orcid":false,"given":"Bernhard","family":"Hellmich","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Rheumatology and Immunology, Medius Kliniken Kirchheim\/Teck, University T\u00fcbingen, Kirchheim-Teck, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1712-0637","authenticated-orcid":false,"given":"David","family":"Jayne","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Cambridge, Cambridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1800-6772","authenticated-orcid":false,"given":"Sara","family":"Monti","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Therapeutics, University of Pavia; Division of Rheumatology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raashid Ahmed","family":"Luqmani","sequence":"additional","affiliation":[{"name":"Oxford NIHR Biomedical Research Centre, Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, University of Oxford, Oxford, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1797-7091","authenticated-orcid":false,"given":"Gunnar","family":"Tomasson","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Iceland, Landspitali University Hospital, Reykjavik, Iceland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2023,7,21]]},"reference":[{"key":"2025090312573263000_9.3.e003082.1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-209133"},{"key":"2025090312573263000_9.3.e003082.2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1803537"},{"key":"2025090312573263000_9.3.e003082.3","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2017-211123"},{"key":"2025090312573263000_9.3.e003082.4","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2023386"},{"key":"2025090312573263000_9.3.e003082.5","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2018-214245"},{"key":"2025090312573263000_9.3.e003082.6","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2021.6615"},{"key":"2025090312573263000_9.3.e003082.7","doi-asserted-by":"crossref","DOI":"10.7326\/L20-1199","article-title":"Long-term rituximab use to maintain remission of antineutrophil cytoplasmic antibody-associated vasculitis","volume":"173","author":"Charles","year":"2020","journal-title":"Ann Intern Med"},{"key":"2025090312573263000_9.3.e003082.8","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-150-10-200905190-00004"},{"key":"2025090312573263000_9.3.e003082.9","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0909905"},{"key":"2025090312573263000_9.3.e003082.10","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0909169"},{"key":"2025090312573263000_9.3.e003082.11","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keu366"},{"key":"2025090312573263000_9.3.e003082.12","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2016-209925"},{"key":"2025090312573263000_9.3.e003082.13","doi-asserted-by":"publisher","DOI":"10.2215\/CJN.11801018"},{"key":"2025090312573263000_9.3.e003082.14","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2017-212878"},{"key":"2025090312573263000_9.3.e003082.15","article-title":"Interventions for renal vasculitis in adults","volume":"1","author":"Walters","year":"2020","journal-title":"Cochrane Database Syst Rev"},{"key":"2025090312573263000_9.3.e003082.16","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1002\/acr2.11230","article-title":"Granulomatosis with polyangiitis and microscopic polyangiitis: a systematic review and meta-analysis of benefits and harms of common treatments","volume":"3","author":"Springer","year":"2021","journal-title":"ACR Open Rheumatol"},{"key":"2025090312573263000_9.3.e003082.17","doi-asserted-by":"crossref","unstructured":"Hellmich B , Sanchez-Alamo B , Schirmer JH , et al . 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