{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T19:24:00Z","timestamp":1790018640101,"version":"4.0.1"},"reference-count":32,"publisher":"BMJ","issue":"3","license":[{"start":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T00:00:00Z","timestamp":1784073600000},"content-version":"unspecified","delay-in-days":14,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2026,6,18]]},"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Avacopan, an oral C5a receptor antagonist, is approved for treatment of severe antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in combination with rituximab or cyclophosphamide. This study evaluated its real-world efficacy, safety and glucocorticoid-sparing effects.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This retrospective single-centre study included 35 patients with new-onset or relapsing AAV treated with avacopan plus standard induction therapy (rituximab, cyclophosphamide or both). Outcomes were compared with 70 matched controls receiving standard therapy. Primary endpoints were remission (Birmingham Vasculitis Activity Score (BVAS)=0, prednisolone \u22645\u2009mg\/day) at 6 and 12 months. Secondary endpoints included relapse, renal function, glucocorticoid exposure and adverse events.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Baseline characteristics were generally comparable, although median estimated glomerular filtration rate (eGFR) was lower in the avacopan group (24 vs 49\u2009mL\/min). Induction therapy included rituximab (28%), cyclophosphamide (11%) or combination therapy (61%), reflecting severe disease. Remission rates were similar at 6 months (69% vs 68%)\u2009but tended to be higher with avacopan at 12 months (86% vs 68%; p=0.11). Relapses occurred less frequently with avacopan (16% vs 51%, p=0.003), including after treatment discontinuation. Avacopan was associated with accelerated glucocorticoid tapering (\u22645\u2009mg\/day; 102 vs 164 days, p&lt;0.001), lower cumulative doses (3266 vs 4288\u2009mg, p=0.008) and greater renal function improvement (\u0394eGFR+18\u2009vs +7\u2009mL\/min after 1 year). Nine patients (26%) discontinued avacopan due to adverse events.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In a real-world practice, avacopan was effective with an acceptable safety profile, enabling steroid sparing, improved renal recovery and reduced relapse rates, including in patients receiving combined induction therapy. Benefits persisted beyond 12 months, but prospective confirmation of these presumed long-term benefits is needed.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/rmdopen-2026-006792","type":"journal-article","created":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T12:10:37Z","timestamp":1784117437000},"page":"e006792","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Real-world efficacy and safety of avacopan in ANCA-associated vasculitis: a retrospective comparative study"],"prefix":"10.1136","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6689-8827","authenticated-orcid":false,"given":"Franz","family":"Thiele","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Medical Center\u2014University of Freiburg, Faculty of Medicine, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johanna","family":"Schneider","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, Medical Center\u2014University of Freiburg, Faculty of Medicine, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric","family":"Prager","sequence":"additional","affiliation":[{"name":"Department of Medicine IV, Medical Center\u2014University of Freiburg, Faculty of Medicine, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilona","family":"Jandova","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Medical Center\u2014University of Freiburg, Faculty of Medicine, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bj\u00f6rn C","family":"Frye","sequence":"additional","affiliation":[{"name":"Department of Pneumology, Medical Center\u2014University of Freiburg, Faculty of Medicine, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reinhard E","family":"Voll","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Medical Center\u2014University of Freiburg, Faculty of Medicine, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3513-3842","authenticated-orcid":false,"given":"Markus A","family":"Schramm","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Medical Center\u2014University of Freiburg, Faculty of Medicine, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7729-2759","authenticated-orcid":false,"given":"Nils","family":"Venhoff","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Medical Center\u2014University of Freiburg, Faculty of Medicine, Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,7,15]]},"reference":[{"key":"2026071505102099000_12.3.e006792.1","unstructured":"Jennette JC , Falk RJ , Bacon PA , et al . Revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 2012;1\u201311."},{"key":"2026071505102099000_12.3.e006792.2","doi-asserted-by":"publisher","DOI":"10.1136\/ard-2022-223764"},{"key":"2026071505102099000_12.3.e006792.3","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keu366"},{"key":"2026071505102099000_12.3.e006792.4","doi-asserted-by":"publisher","DOI":"10.1681\/ASN.2016111179"},{"key":"2026071505102099000_12.3.e006792.5","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1002\/acr2.11185","article-title":"Adjunctive treatment with avacopan, an oral C5a receptor inhibitor, in patients with antineutrophil cytoplasmic antibody-associated vasculitis","volume":"2","author":"Merkel","year":"2020","journal-title":"ACR Open Rheumatology"},{"key":"2026071505102099000_12.3.e006792.6","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2023386"},{"key":"2026071505102099000_12.3.e006792.7","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1016\/j.ekir.2023.01.039","article-title":"Renal recovery for patients with ANCA-associated vasculitis and low eGFR in the ADVOCATE trial of avacopan","volume":"8","author":"Cortazar","year":"2023","journal-title":"Kidney Int Rep"},{"key":"2026071505102099000_12.3.e006792.8","doi-asserted-by":"crossref","first-page":"2751","DOI":"10.1016\/j.ekir.2025.05.041","article-title":"Treatment with avacopan in ANCA-associated vasculitis with kidney involvement","volume":"10","author":"Geetha","year":"2025","journal-title":"Kidney Int Rep"},{"key":"2026071505102099000_12.3.e006792.9","doi-asserted-by":"publisher","DOI":"10.3349\/ymj.2022.0435"},{"key":"2026071505102099000_12.3.e006792.10","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-150-10-200905190-00004"},{"key":"2026071505102099000_12.3.e006792.11","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2008.101279"},{"key":"2026071505102099000_12.3.e006792.12","first-page":"75","article-title":"Methods of estimating GFR\u2014different equations including CKD-EPI","volume":"32","author":"Florkowski","year":"2011","journal-title":"Clin Biochem Rev"},{"key":"2026071505102099000_12.3.e006792.13","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2006.062711"},{"key":"2026071505102099000_12.3.e006792.14","doi-asserted-by":"crossref","first-page":"4868","DOI":"10.1093\/rheumatology\/keaa924","article-title":"New insights into the epidemiology of ANCA-associated vasculitides in Germany: results from a claims data study","volume":"60","author":"Hellmich","year":"2021","journal-title":"Rheumatology"},{"key":"2026071505102099000_12.3.e006792.15","doi-asserted-by":"crossref","first-page":"1473","DOI":"10.1093\/ndt\/gfae020","article-title":"Avacopan for ANCA-associated vasculitis with hypoxic pulmonary haemorrhage","volume":"39","author":"Chalkia","year":"2024","journal-title":"Nephrol Dial Transplant"},{"key":"2026071505102099000_12.3.e006792.16","doi-asserted-by":"crossref","first-page":"2019","DOI":"10.1093\/rheumatology\/keae534","article-title":"The real-world use and effectiveness of avacopan in routine practice for the treatment of ANCA vasculitis: first experiences in Spain","volume":"64","author":"Draibe","year":"2025","journal-title":"Rheumatology (Oxford)"},{"key":"2026071505102099000_12.3.e006792.17","doi-asserted-by":"crossref","first-page":"1783","DOI":"10.1016\/j.ekir.2024.03.022","article-title":"Real-world experience with avacopan in antineutrophil cytoplasmic autoantibody-associated vasculitis","volume":"9","author":"Zonozi","year":"2024","journal-title":"Kidney Int Rep"},{"key":"2026071505102099000_12.3.e006792.18","doi-asserted-by":"publisher","DOI":"10.1002\/art.11115"},{"key":"2026071505102099000_12.3.e006792.19","doi-asserted-by":"crossref","first-page":"2338","DOI":"10.1002\/art.40313","article-title":"The epidemiology of antineutrophil cytoplasmic autoantibody-associated vasculitis in Olmsted County, Minnesota","volume":"69","author":"Berti","year":"2017","journal-title":"Arthritis Rheumatol"},{"key":"2026071505102099000_12.3.e006792.20","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0909169"},{"key":"2026071505102099000_12.3.e006792.21","doi-asserted-by":"crossref","first-page":"394","DOI":"10.1016\/j.ekir.2017.11.004","article-title":"Combination therapy with rituximab and cyclophosphamide for remission induction in ANCA vasculitis","volume":"3","author":"Cortazar","year":"2018","journal-title":"Kidney Int Rep"},{"key":"2026071505102099000_12.3.e006792.22","doi-asserted-by":"crossref","first-page":"1316","DOI":"10.1016\/j.kint.2021.08.025","article-title":"Combination treatment with rituximab, low-dose cyclophosphamide and plasma exchange for severe antineutrophil cytoplasmic antibody-associated vasculitis","volume":"100","author":"Gulati","year":"2021","journal-title":"Kidney Int"},{"key":"2026071505102099000_12.3.e006792.23","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2023.10.008"},{"key":"2026071505102099000_12.3.e006792.24","doi-asserted-by":"crossref","first-page":"2214","DOI":"10.1093\/rheumatology\/keae359","article-title":"Avacopan for anti-neutrophil cytoplasm antibodies-associated vasculitis: a multicentre real-world study","volume":"64","author":"Gabilan","year":"2025","journal-title":"Rheumatology (Oxford)"},{"key":"2026071505102099000_12.3.e006792.25","doi-asserted-by":"crossref","unstructured":"Patel NJ , King A , Negron M , et al . Real-world clinical outcomes in a large health care system after avacopan initiation for granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) involving the kidneys. J Am Soc Nephrol 2025;36. doi:10.1681\/ASN.2025sh98amsv","DOI":"10.1681\/ASN.2025sh98amsv"},{"key":"2026071505102099000_12.3.e006792.26","doi-asserted-by":"crossref","first-page":"2803","DOI":"10.1016\/j.ekir.2024.07.007","article-title":"Avacopan in anti-neutrophil cytoplasmic autoantibodies-associated vasculitis in a real-world setting","volume":"9","author":"Zimmermann","year":"2024","journal-title":"Kidney Int Rep"},{"key":"2026071505102099000_12.3.e006792.27","doi-asserted-by":"crossref","DOI":"10.1186\/s41927-025-00555-2","article-title":"Avacopan is effective in inducing remission for MPA\/GPA, regardless of changes in serum C5a levels: a single-center study in Japan","volume":"9","author":"Ushio","year":"2025","journal-title":"BMC Rheumatol"},{"key":"2026071505102099000_12.3.e006792.28","doi-asserted-by":"crossref","first-page":"2646","DOI":"10.1093\/rheumatology\/kead087","article-title":"CanVasc consensus recommendations for the use of avacopan in antineutrophil cytoplasm antibody-associated vasculitis: 2022 addendum","volume":"62","author":"Turgeon","year":"2023","journal-title":"Rheumatology (Oxford)"},{"key":"2026071505102099000_12.3.e006792.29","doi-asserted-by":"crossref","first-page":"1115","DOI":"10.1016\/j.ekir.2022.01.1065","article-title":"Avacopan as first-line treatment in antineutrophil cytoplasmic antibody-associated vasculitis: a steroid-sparing option","volume":"7","author":"Gabilan","year":"2022","journal-title":"Kidney Int Rep"},{"key":"2026071505102099000_12.3.e006792.30","doi-asserted-by":"crossref","first-page":"338","DOI":"10.5414\/CN111775","article-title":"Avacopan for ANCA-associated vasculitis: Beyond the 52-week treatment course","volume":"104","author":"Eisinger","year":"2025","journal-title":"CN"},{"key":"2026071505102099000_12.3.e006792.31","doi-asserted-by":"crossref","first-page":"2533","DOI":"10.1093\/rheumatology\/keae689","article-title":"Drug-induced liver injury related to avacopan therapy","volume":"64","author":"Mori","year":"2025","journal-title":"Rheumatology (Oxford)"},{"key":"2026071505102099000_12.3.e006792.32","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2014.11.009"}],"container-title":["RMD Open"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/rmdopen-2026-006792","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T12:10:45Z","timestamp":1784117445000},"score":1,"resource":{"primary":{"URL":"https:\/\/rmdopen.bmj.com\/lookup\/doi\/10.1136\/rmdopen-2026-006792"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":32,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2026,7,15]]},"published-print":{"date-parts":[[2026,7]]}},"alternative-id":["10.1136\/rmdopen-2026-006792"],"URL":"https:\/\/doi.org\/10.1136\/rmdopen-2026-006792","relation":{},"ISSN":["2056-5933"],"issn-type":[{"value":"2056-5933","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]}}}