{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,18]],"date-time":"2026-04-18T21:32:07Z","timestamp":1776547927774,"version":"3.51.2"},"reference-count":14,"publisher":"Springer Science and Business Media LLC","issue":"2","license":[{"start":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T00:00:00Z","timestamp":1770076800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T00:00:00Z","timestamp":1770076800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["CEN Case Rep"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-positive vasculitis frequently affects the kidney and the lung, with alveolar hemorrhage being fatal. Whereas aggressive immunosuppressive therapies are conventionally used, recent studies have shown the beneficial effect of avacopan, a C5a antagonist, as an alternative to glucocorticoids for ANCA-associated vasculitis (AAV). In patients with pulmonary hemorrhage and severe respiratory failure, however, neither the efficacy of avacopan nor the contribution of this drug to early withdrawal of glucocorticoids is fully qualified. Here, we report a case of AAV presenting with alveolar hemorrhage requiring aggressive ventilatory support in which we experienced the favorable effect of early use of avacopan. A 31-year-old man was referred to our hospital because of a two-week history of blood sputum and positive MPO-ANCA. His respiratory failure deteriorated rapidly, necessitating both mechanical ventilation and extracorporeal membrane oxygenation. A combination therapy with glucocorticoids and rituximab was initiated and avacopan was started on hospital day 8, which resulted in successful remission within six months of admission (Birmingham Vasculitis Activity Score version 3\u2009=\u20090), and the beneficial effect was sustained for at least 6\u00a0months following the discontinuation of glucocorticoid withdrawal (day 156). Thus, avacopan, in combination with immunosuppressives, may not only help suppress the disease activity of AAV but also facilitate early withdrawal of glucocorticoids even in case of life-threatening respiratory failure.<\/jats:p>","DOI":"10.1007\/s13730-025-01068-0","type":"journal-article","created":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T03:05:38Z","timestamp":1770087938000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Avacopan for severe pulmonary hemorrhage requiring extracorporeal membrane oxygenation in a patient with MPO-ANCA positive vasculitis"],"prefix":"10.1007","volume":"15","author":[{"given":"Keita","family":"Endo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koichi","family":"Hayashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuki","family":"Hara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akihiro","family":"Miyake","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keisuke","family":"Takano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kaede","family":"Yoshino","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koichi","family":"Kitamura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shinsuke","family":"Ito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shigeki","family":"Fujitani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4545-9769","authenticated-orcid":false,"given":"Toshihiko","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,2,3]]},"reference":[{"issue":"7","key":"1068_CR1","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1056\/NEJMoa2023386","volume":"384","author":"DRW Jayne","year":"2021","unstructured":"Jayne DRW, Merkel PA, Schall TJ, Bekker P. Avacopan for the treatment of ANCA-associated vasculitis. N Engl J Med. 2021;384(7):599\u2013609. https:\/\/doi.org\/10.1056\/NEJMoa2023386.","journal-title":"N Engl J Med"},{"issue":"9","key":"1068_CR2","doi-asserted-by":"publisher","first-page":"1473","DOI":"10.1093\/ndt\/gfae020","volume":"39","author":"A Chalkia","year":"2024","unstructured":"Chalkia A, Flossmann O, Jones R, Nair JR, Simpson T, Smith R, et al. Avacopan for ANCA-associated vasculitis with hypoxic pulmonary haemorrhage. Nephrol Dial Transplant. 2024;39(9):1473\u201382. https:\/\/doi.org\/10.1093\/ndt\/gfae020.","journal-title":"Nephrol Dial Transplant"},{"key":"1068_CR3","doi-asserted-by":"publisher","DOI":"10.1002\/acr2.11726","author":"SD Falde","year":"2024","unstructured":"Falde SD, Lal A, Cartin-Ceba R, Mertz LE, Fervenza FC, Zand L, et al. Treatment of antineutrophil cytoplasmic antibody-associated vasculitis with diffuse alveolar hemorrhage with avacopan. ACR Open Rheumatol. 2024. https:\/\/doi.org\/10.1002\/acr2.11726.","journal-title":"ACR Open Rheumatol"},{"issue":"2","key":"1068_CR4","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1038\/s41584-018-0145-y","volume":"15","author":"D Nakazawa","year":"2019","unstructured":"Nakazawa D, Masuda S, Tomaru U, Ishizu A. Pathogenesis and therapeutic interventions for ANCA-associated vasculitis. Nat Rev Rheumatol. 2019;15(2):91\u2013101. https:\/\/doi.org\/10.1038\/s41584-018-0145-y.","journal-title":"Nat Rev Rheumatol"},{"issue":"4","key":"1068_CR5","doi-asserted-by":"publisher","first-page":"277","DOI":"10.1007\/s13730-023-00841-3","volume":"13","author":"H Miyake","year":"2024","unstructured":"Miyake H, Tanabe K, Yamaji S, Kihara T. Early transition to avacopan from glucocorticoids applied during induction therapy for microscopic polyangiitis with rapidly progressive glomerulonephritis. CEN Case Rep. 2024;13(4):277\u201383. https:\/\/doi.org\/10.1007\/s13730-023-00841-3.","journal-title":"CEN Case Rep"},{"key":"1068_CR6","doi-asserted-by":"publisher","DOI":"10.1007\/s13730-024-00929-4","author":"R Tanaka","year":"2024","unstructured":"Tanaka R, Toishi T, Masaki R, Aihara H, Sakamoto S, Ikeda M, et al. Effective management of necrotizing crescentic glomerulonephritis using an aggressive combination therapy including avacopan in a patient double-seropositive for anti-GBM antibodies and ANCA: a case report. CEN Case Rep. 2024. https:\/\/doi.org\/10.1007\/s13730-024-00929-4.","journal-title":"CEN Case Rep"},{"issue":"1","key":"1068_CR7","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1007\/s13730-024-00910-1","volume":"14","author":"Y Ubara","year":"2025","unstructured":"Ubara Y, Oba Y, Kurihara S, Sekine A, Yamanouchi M, Hasegawa E, et al. A case of renal limited myeloperoxidase anti-neutrophil cytoplasmic antibody-positive vasculitis treated with maintenance avacopan monotherapy. CEN Case Rep. 2025;14(1):85\u20139. https:\/\/doi.org\/10.1007\/s13730-024-00910-1.","journal-title":"CEN Case Rep"},{"key":"1068_CR8","doi-asserted-by":"publisher","DOI":"10.1007\/s13730-025-00965-8","author":"T Tsuchiya","year":"2025","unstructured":"Tsuchiya T, Ohta Y, Oka M, Fukushima K, Matsuno S, Yamanaka N, et al. Successful use of avacopan in a case of ANCA-associated vasculitis with treatment-resistant medium-sized vessel involvement. CEN Case Rep. 2025. https:\/\/doi.org\/10.1007\/s13730-025-00965-8.","journal-title":"CEN Case Rep"},{"issue":"Suppl 1","key":"1068_CR9","doi-asserted-by":"publisher","first-page":"i83","DOI":"10.1093\/ndt\/gfu385","volume":"30","author":"K Hirayama","year":"2015","unstructured":"Hirayama K, Kobayashi M, Usui J, Arimura Y, Sugiyama H, Nitta K, et al. Pulmonary involvements of anti-neutrophil cytoplasmic autoantibody-associated renal vasculitis in Japan. Nephrol Dial Transplant. 2015;30(Suppl 1):i83-93. https:\/\/doi.org\/10.1093\/ndt\/gfu385.","journal-title":"Nephrol Dial Transplant"},{"issue":"3","key":"1068_CR10","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1055\/s-0031-1279830","volume":"32","author":"A Casian","year":"2011","unstructured":"Casian A, Jayne D. Management of alveolar hemorrhage in lung vasculitides. Semin Respir Crit Care Med. 2011;32(3):335\u201345. https:\/\/doi.org\/10.1055\/s-0031-1279830.","journal-title":"Semin Respir Crit Care Med"},{"key":"1068_CR11","doi-asserted-by":"publisher","DOI":"10.3390\/jcm13226676","author":"J Sandino","year":"2024","unstructured":"Sandino J, Morales E. Role of avacopan on antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. J Clin Med. 2024. https:\/\/doi.org\/10.3390\/jcm13226676.","journal-title":"J Clin Med"},{"issue":"2","key":"1068_CR12","doi-asserted-by":"publisher","first-page":"338","DOI":"10.1093\/mr\/roac037","volume":"33","author":"M Harigai","year":"2023","unstructured":"Harigai M, Kaname S, Tamura N, Dobashi H, Kubono S, Yoshida T. Efficacy and safety of avacopan in Japanese patients with antineutrophil cytoplasmic antibody-associated vasculitis: a subanalysis of a randomized Phase 3 study. Mod Rheumatol. 2023;33(2):338\u201345. https:\/\/doi.org\/10.1093\/mr\/roac037.","journal-title":"Mod Rheumatol"},{"issue":"6","key":"1068_CR13","doi-asserted-by":"publisher","first-page":"944","DOI":"10.1093\/ndt\/gfad237","volume":"39","author":"A Chalkia","year":"2024","unstructured":"Chalkia A, Jayne D. ANCA-associated vasculitis-treatment standard. Nephrol Dial Transplant. 2024;39(6):944\u201355. https:\/\/doi.org\/10.1093\/ndt\/gfad237.","journal-title":"Nephrol Dial Transplant"},{"issue":"6","key":"1068_CR14","doi-asserted-by":"publisher","first-page":"1783","DOI":"10.1016\/j.ekir.2024.03.022","volume":"9","author":"R Zonozi","year":"2024","unstructured":"Zonozi R, Aqeel F, Le D, Cortazar FB, Thaker J, Zabala Ramirez MJ, et al. Real-world experience with Avacopan in antineutrophil cytoplasmic autoantibody-associated vasculitis. Kidney Int Rep. 2024;9(6):1783\u201391. https:\/\/doi.org\/10.1016\/j.ekir.2024.03.022.","journal-title":"Kidney Int Rep"}],"container-title":["CEN Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s13730-025-01068-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s13730-025-01068-0","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s13730-025-01068-0.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T03:05:42Z","timestamp":1770087942000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s13730-025-01068-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,3]]},"references-count":14,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,4]]}},"alternative-id":["1068"],"URL":"https:\/\/doi.org\/10.1007\/s13730-025-01068-0","relation":{},"ISSN":["2192-4449"],"issn-type":[{"value":"2192-4449","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,3]]},"assertion":[{"value":"2 May 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 September 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"3 February 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors have declared that no conflict of interest exists.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and\/or national research committee at which the studies were conducted (IRB approval number 1019) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}},{"value":"The patient gave a written informed consent for the treatment and the publication of this case report.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}}],"article-number":"39"}}