{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T15:02:29Z","timestamp":1784732549008,"version":"3.55.0"},"reference-count":14,"publisher":"BMJ","issue":"7","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2026,7,6]]},"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:p>Avacopan, an oral C5a receptor 1 antagonist, is approved as a glucocorticoid-sparing option for remission induction in antineutrophil cytoplasmic antibody-associated vasculitis. Since April 2026, its marketing authorisation is undergoing regulatory review by the US Food and Drug Administration and the European Medicines Agency. We report the case of a middle-aged man with granulomatosis with polyangiitis (GPA) presenting with severe gastrointestinal involvement complicated by recurrent bleeding and bowel perforations together with rapidly progressive glomerulonephritis. Despite rituximab and high-dose glucocorticoids, disease activity progressed. Avacopan was introduced as adjunctive therapy during critical illness. Its initiation was temporally associated with multi-organ recovery and enabled rapid corticosteroid withdrawal, with no evidence of hepatotoxicity. This case illustrates real-world use of avacopan in a severe multisystem GPA phenotype with gastrointestinal involvement and adds to the accumulating post-marketing clinical experience being evaluated in ongoing regulatory assessments of this agent.<\/jats:p>","DOI":"10.1136\/bcr-2026-274337","type":"journal-article","created":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T14:20:26Z","timestamp":1784730026000},"page":"e274337","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Use of avacopan in granulomatosis with polyangiitis with predominant gastrointestinal and kidney involvement"],"prefix":"10.1136","volume":"19","author":[{"given":"Pone","family":"De Weerdt","sequence":"first","affiliation":[{"name":"Department of Nephrology and Hypertension, Universitair Ziekenhuis Brussel, Brussels, Belgium"},{"name":"Vrije Universiteit Brussel, Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ward","family":"Janssens","sequence":"additional","affiliation":[{"name":"Vrije Universiteit Brussel, Brussels, Belgium"},{"name":"Department of Abdominal Surgery, Universitair Ziekenhuis Brussel, Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5437-1689","authenticated-orcid":false,"given":"Michael","family":"Mekeirele","sequence":"additional","affiliation":[{"name":"Vrije Universiteit Brussel, Brussels, Belgium"},{"name":"Department of Intensive Care Medicine, Universitair Ziekenhuis Brussel, Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tim","family":"Balthazar","sequence":"additional","affiliation":[{"name":"Vrije Universiteit Brussel, Brussels, Belgium"},{"name":"Department of Cardiology, Universitair Ziekenhuis Brussel, Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kasper","family":"Hermans","sequence":"additional","affiliation":[{"name":"Department of Rheumatic and Systemic Diseases, Universitair Ziekenhuis Brussel, Brussels, Belgium"},{"name":"Vitality Research Group, Vrije Universiteit Brussel (VUB), Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Celeste","family":"Smeys","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Hypertension, Universitair Ziekenhuis Brussel, Brussels, Belgium"},{"name":"Vrije Universiteit Brussel, Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0981-8621","authenticated-orcid":false,"given":"Peter","family":"Janssens","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Hypertension, Universitair Ziekenhuis Brussel, Brussels, Belgium"},{"name":"Vitality Research Group, Vrije Universiteit Brussel (VUB), Brussels, Belgium"},{"name":"Kidney Diseases, Dialysis & Transplantation Research Unit (NIER), Vrije Universiteit Brussel, Brussels, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2026,7,22]]},"reference":[{"key":"2026072207202069000_19.7.e274337.1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(23)01736-1"},{"key":"2026072207202069000_19.7.e274337.2","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.170249"},{"key":"2026072207202069000_19.7.e274337.3","doi-asserted-by":"crossref","DOI":"10.1186\/s41927-025-00585-w","article-title":"Gastrointestinal involvement in granulomatosis with polyangiitis: frequency, clinical impact, and prognostic implications in a retrospective cohort study","volume":"9","author":"Siri","year":"2025","journal-title":"BMC Rheumatol"},{"key":"2026072207202069000_19.7.e274337.4","first-page":"270","article-title":"Gastrointestinal tract involvement in granulomatosis with polyangiitis","volume":"11","author":"Masiak","year":"2016","journal-title":"Prz Gastroenterol"},{"key":"2026072207202069000_19.7.e274337.5","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2024.1409129","article-title":"Treatment goals in ANCA-associated vasculitis: defining success in a new era","volume":"15","author":"Alberici","year":"2024","journal-title":"Front Immunol"},{"key":"2026072207202069000_19.7.e274337.6","doi-asserted-by":"publisher","DOI":"10.1016\/j.kint.2023.10.008"},{"key":"2026072207202069000_19.7.e274337.7","doi-asserted-by":"publisher","DOI":"10.1136\/ard-2022-223764"},{"key":"2026072207202069000_19.7.e274337.8","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2023386"},{"key":"2026072207202069000_19.7.e274337.9","unstructured":"U.S. Food and Drug Administration . FDA identifies cases of serious liver injury in patients taking Tavneos (avacopan) for severe active anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis. Silver Spring (MD): FDA; 2026. Available: https:\/\/www.fda.gov\/drugs\/drug-safety-communications\/fda-identifies-cases-serious-liver-injury-patients-taking-tavneos-avacopan-severe-active-anti"},{"key":"2026072207202069000_19.7.e274337.10","unstructured":"U.S. Food and Drug Administration . Proposal to withdraw marketing approval; notice of opportunity for a hearing: TAVNEOS (avacopan) capsule, 10 mg, NDA 214487. Silver Spring (MD): FDA; 2026. Available: https:\/\/www.fda.gov\/media\/192160\/download?attachment"},{"key":"2026072207202069000_19.7.e274337.11","unstructured":"European Medicines Agency . EMA starts review of Tavneos, a medicine for rare autoimmune diseases GPA and MPA. Amsterdam: EMA; 2026. Available: https:\/\/www.ema.europa.eu\/en\/news\/ema-starts-review-tavneos-medicine-rare-autoimmune-diseases-gpa-mpa"},{"key":"2026072207202069000_19.7.e274337.12","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":"2026072207202069000_19.7.e274337.13","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1803537"},{"key":"2026072207202069000_19.7.e274337.14","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"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2026-274337","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,22]],"date-time":"2026-07-22T14:20:29Z","timestamp":1784730029000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2026-274337"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":14,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2026,7,22]]},"published-print":{"date-parts":[[2026,7]]}},"alternative-id":["10.1136\/bcr-2026-274337"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2026-274337","relation":{},"ISSN":["1757-790X"],"issn-type":[{"value":"1757-790X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]}}}