{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T05:46:51Z","timestamp":1791784011036,"version":"4.3.4"},"reference-count":10,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T00:00:00Z","timestamp":1791676800000},"content-version":"vor","delay-in-days":10,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T00:00:00Z","timestamp":1790812800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ACR Open Rheumatology"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Complement activation, particularly through the C5a\u2013C5a receptor axis, plays a pivotal role in the pathogenesis of antineutrophil cytoplasmic antibody\u2013associated vasculitis (AAV) and may represent an important therapeutic target. Avacopan, an oral C5a receptor antagonist, was the first therapy targeting this pathway to enter in clinical practice. Prospective real\u2010world data on its long\u2010term effectiveness, safety, and impact on quality of life (QoL) remain limited. In the context of an evolving therapeutic and regulatory landscape, such data provide valuable evidence on the clinical effects of C5a receptor inhibition and may inform the development and evaluation of future complement\u2010targeted therapies. The objective of this study was to evaluate the real\u2010world effectiveness, safety, kidney outcomes, and patient\u2010reported outcomes of avacopan in an Italian cohort of patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This prospective, multicenter, observational study included patients with newly diagnosed or relapsing GPA or MPA who were started on avacopan therapy across 18 Italian centers. Avacopan was administered in combination with standard immunosuppressive therapy, with or without glucocorticoids (GCs). Clinical remission (Birmingham Vasculitis Activity Score\u00a0=\u00a00), GC\u2010free remission, kidney parameters, laboratory outcomes, adverse events, and patient\u2010reported outcomes assessed by the AAV Patient\u2010Reported Outcome questionnaire were evaluated up to 52 weeks and beyond. Estimated glomerular filtration rate (eGFR) was calculated using the European Kidney Function Consortium equation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Forty\u2010six patients were included (63% women; mean age of 59 years); 61% had relapsing disease, and 70% had kidney involvement at baseline. Clinical remission was achieved by 78%, 88%, and 97% of patients at weeks 12, 26, and 52, respectively. GC\u2010free remission occurred in 20% at week 12 and 55% at week 52. Mean GC dose decreased from 23 mg\/day at baseline to 5 mg\/day by week 12 (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.001). Kidney function improved over time, with median eGFR increasing from 30.5 mL\/min\/1.73 m\n                      <jats:sup>2<\/jats:sup>\n                      at baseline to 53.5 mL\/min\/1.73 m\n                      <jats:sup>2<\/jats:sup>\n                      at week 52 (\n                      <jats:italic>P<\/jats:italic>\n                      \u00a0=\u00a00.002). Significant improvements in QoL were observed as early as week 12 and were sustained until week 52, particularly in the physical function domain. Adverse events were mild to moderate; infections were the most frequent events (40%). Disease relapses were uncommon and occurred exclusively in patients treated for relapsing disease.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In this real\u2010world Italian cohort, patients treated with avacopan achieved high remission rates, substantial GC reduction, improvement in kidney function, and sustained QoL benefits. These findings provide prospective real\u2010world evidence on the clinical outcomes associated with C5a receptor inhibition in routine clinical practice while acknowledging the observational design of the study.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/acr2.90126","type":"journal-article","created":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T05:37:59Z","timestamp":1791783479000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Real\u2010World Use of Avacopan in Antineutrophil Cytoplasmic Antibody\u2013Associated Vasculitis: Early and Long\u2010Term Clinical, Renal, and Patient\u2010Reported Outcomes in an Italian Rheumatology\u2013Nephrology Network"],"prefix":"10.1002","volume":"8","author":[{"given":"Luca","family":"Quartuccio","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Medicine University of Udine  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Treppo","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Medicine University of Udine  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam","family":"Isola","sequence":"additional","affiliation":[{"name":"Department of Medical Area, Institute of Statistics University of Udine  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"De Martino","sequence":"additional","affiliation":[{"name":"Department of Medical Area, Institute of Statistics University of Udine  Udine Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7027-1410","authenticated-orcid":false,"given":"Chiara","family":"Marvisi","sequence":"additional","affiliation":[{"name":"Azienda USL\u2010IRCCS di Reggio Emilia and University of Modena and Reggio Emilia  Reggio Emilia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Iorio","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Medicine DIMED University of Padua  Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"Milanesi","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine and Therapeutics, IRCCS Policlinico San Matteo University of Pavia  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Moroni","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) IRCCS San Raffaele Scientific Institute  Milano Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edoardo","family":"Conticini","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Medicine, Surgery and Neurosciences University of Siena  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Department of Biomedical and Clinical Sciences Sacco and Fatebenefratelli Hospitals  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pasquale","family":"Esposito","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Medical Specialties (DIMI) University of Genoa  Genova Italy"},{"name":"Unit of Nephrology, Dialysis and Transplantation, IRCCS Azienda Ospedaliera Metropolitana Policlinico San Martino  Genova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0474-5344","authenticated-orcid":false,"given":"Florenzo","family":"Iannone","sequence":"additional","affiliation":[{"name":"DiMePReJ \u2010 Rheumatology Unit University of Bari  Bari Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Binda","sequence":"additional","affiliation":[{"name":"Department of Nephrology, Dialysis and Kidney Transplantation Fondazione IRCCS Ca\u2019 Granda 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Meyer Children's Hospital IRCCS  Florence Italy"},{"name":"Department of Biomedical, Experimental and Clinical Science University of Florence  Florence Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bruno","family":"Frediani","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Medicine, Surgery and Neurosciences University of Siena  Siena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Delvino","sequence":"additional","affiliation":[{"name":"School of Medicine and Surgery, University of Milano\u2010Bicocca  Milan Italy"},{"name":"Rheumatology Unit Fondazione IRCCS San Gerardo dei Tintori  Monza Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1800-6772","authenticated-orcid":false,"given":"Sara","family":"Monti","sequence":"additional","affiliation":[{"name":"IRCCS Istituto Auxologico Italiano, Unit of Immunology, Allergology and Rheumatology  Milan Italy"},{"name":"Department of Clinical Sciences and Community Health University of Milan  Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2356-375X","authenticated-orcid":false,"given":"Roberto","family":"Padoan","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Medicine DIMED University of Padua  Padova Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlomaurizio","family":"Montecucco","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine and Therapeutics, IRCCS Policlinico San Matteo University of Pavia  Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Dagna","sequence":"additional","affiliation":[{"name":"Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR) IRCCS San Raffaele Scientific Institute  Milano 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cases of serious liver injury in patients taking tavneos (avacopan) for severe active anti\u2010neutrophil cytoplasmic autoantibody (ANCA)\u2010associated vasculitis","year":"2026"}],"container-title":["ACR Open 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