{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,11]],"date-time":"2025-09-11T16:53:35Z","timestamp":1757609615828,"version":"3.44.0"},"reference-count":27,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2025,5,27]],"date-time":"2025-05-27T00:00:00Z","timestamp":1748304000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,9,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Polyangiitis overlap syndrome, characterized by the coexistence of giant cell arteritis (GCA) and anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), is a rare and poorly understood condition. Clinical features, treatment responses, and long-term prognosis of this entity remain unclear. This study aimed to elucidate clinical characteristics, relapse patterns, and potential therapeutic strategies for polyangiitis overlap syndrome.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>We retrospectively analysed consecutive patients with GCA or AAV at our institution between January 2012 and September 2024 and identified cases of polyangiitis overlap syndrome. Clinical data, including symptoms, laboratory findings, treatment regimens, and outcomes, were extracted from medical records and analysed descriptively.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>Among 60 GCA and 151 AAV cases, we identified six cases of polyangiitis overlap syndrome. The median age at onset was 68\u00a0years (range: 54\u201375), and 33% were female. Microscopic polyangiitis was the most common AAV subtype (50%). Fever was the predominant symptom (83%), and kidney involvement was observed in 83%, followed by pulmonary involvement (67%). All patients received high-dose glucocorticoids; four received cyclophosphamide, and two received tocilizumab as induction therapy. Over a median follow-up of 32\u00a0months (range: 5\u2013122), four of six patients (67%) experienced relapse, all due to AAV-related activity. Notably, two patients who received tocilizumab as maintenance therapy remained in remission with minimal glucocorticoid exposure.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Polyangiitis overlap syndrome represents a high-risk phenotype for relapse, driven by AAV activity. Our findings suggest that IL-6 receptor blockade is a potential therapeutic option, highlighting the need for further research to establish optimal maintenance strategies in this complex disease entity.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/mr\/roaf041","type":"journal-article","created":{"date-parts":[[2025,5,27]],"date-time":"2025-05-27T07:01:59Z","timestamp":1748329319000},"page":"878-883","source":"Crossref","is-referenced-by-count":0,"title":["Polyangiitis overlap syndrome is a high-risk clinical phenotype for relapse: Case series from the KEIO-vasculitis cohort"],"prefix":"10.1093","volume":"35","author":[{"given":"Hiroki","family":"Tabata","sequence":"first","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5075-8977","authenticated-orcid":false,"given":"Mitsuhiro","family":"Akiyama","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuki","family":"Imai","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koji","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kanako","family":"Shimanuki","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koichi","family":"Saito","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroshi","family":"Takei","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasushi","family":"Kondo","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun","family":"Kikuchi","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuko","family":"Kaneko","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2025,5,27]]},"reference":[{"key":"2025090306271928800_ref1","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1016\/0002-9343(86)90186-5","article-title":"Polyangiitis overlap syndrome. Classification and prospective clinical experience","volume":"81","author":"Leavitt","year":"1986","journal-title":"Am J Med"},{"key":"2025090306271928800_ref2","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/0002-9343(84)90448-0","article-title":"Giant cell arteritis with pulmonary nodules","volume":"77","author":"Bradley","year":"1984","journal-title":"Am J Med"},{"key":"2025090306271928800_ref3","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/S0025-6196(12)60157-8","article-title":"Wegener's granulomatosis associated with vasculitis of the temporal artery: report of five cases","volume":"68","author":"Nishino","year":"1993","journal-title":"Mayo Clin Proc"},{"key":"2025090306271928800_ref4","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1080\/24725625.2020.1780003","article-title":"A case of polyangiitis overlap syndrome of giant cell arteritis and granulomatosis with polyangiitis successfully treated with rituximab","volume":"5","author":"Shibata","year":"2021","journal-title":"Mod Rheumatol Case Rep"},{"key":"2025090306271928800_ref5","doi-asserted-by":"crossref","first-page":"651","DOI":"10.12659\/AJCR.909243","article-title":"Co-presentation of giant cell arteritis and granulomatosis with polyangiitis: a case report and review of literature","volume":"19","author":"Hassane","year":"2018","journal-title":"Am J Case Rep"},{"key":"2025090306271928800_ref6","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1056\/NEJMoa1613849","article-title":"Trial of tocilizumab in giant-cell arteritis","volume":"377","author":"Stone","year":"2017","journal-title":"N Engl J Med"},{"key":"2025090306271928800_ref7","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1007\/s00296-022-05217-x","article-title":"Giant cell arteritis successfully treated with subcutaneous tocilizumab monotherapy","volume":"43","author":"Higashida-Konishi","year":"2023","journal-title":"Rheumatol Int"},{"key":"2025090306271928800_ref8","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1056\/NEJMoa0909905","article-title":"Rituximab versus cyclophosphamide for ANCA-associated vasculitis","volume":"363","author":"Stone","year":"2010","journal-title":"N Engl J Med"},{"key":"2025090306271928800_ref9","doi-asserted-by":"crossref","first-page":"102737","DOI":"10.1016\/j.autrev.2020.102737","article-title":"Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis: a systematic literature review","volume":"20","author":"Akiyama","year":"2021","journal-title":"Autoimmun Rev"},{"key":"2025090306271928800_ref10","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1056\/NEJMoa2023386","article-title":"Avacopan for the treatment of ANCA-associated vasculitis","volume":"384","author":"DRW","year":"2021","journal-title":"N Engl J Med"},{"key":"2025090306271928800_ref11","doi-asserted-by":"crossref","first-page":"1647","DOI":"10.1136\/ard-2022-223480","article-title":"2022 American College of Rheumatology\/EULAR classification criteria for giant cell arteritis","volume":"81","author":"Ponte","year":"2022","journal-title":"Ann Rheum Dis"},{"key":"2025090306271928800_ref12","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1136\/annrheumdis-2021-221796","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis","volume":"81","author":"Suppiah","year":"2022","journal-title":"Ann Rheum Dis"},{"key":"2025090306271928800_ref13","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1136\/annrheumdis-2021-221795","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis","volume":"81","author":"Robson","year":"2022","journal-title":"Ann Rheum Dis"},{"key":"2025090306271928800_ref14","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1136\/annrheumdis-2021-221794","article-title":"2022 American College of Rheumatology\/European Alliance of Associations for Rheumatology Classification criteria for eosinophilic granulomatosis with polyangiitis","volume":"81","author":"Grayson","year":"2022","journal-title":"Ann Rheum Dis"},{"key":"2025090306271928800_ref15","doi-asserted-by":"crossref","first-page":"1102","DOI":"10.3899\/jrheum.2024-0335","article-title":"Performance of the 2022 ACR\/EULAR classification criteria in comparison with the European medicines agency algorithm in antineutrophil cytoplasmic antibody-associated vasculitis","volume":"51","author":"Imai","year":"2024","journal-title":"J Rheumatol"},{"key":"2025090306271928800_ref16","doi-asserted-by":"crossref","first-page":"521","DOI":"10.3899\/jrheum.170508","article-title":"Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis in Japan: a nationwide, prospective cohort study","volume":"45","author":"Hara","year":"2018","journal-title":"J Rheumatol"},{"key":"2025090306271928800_ref17","doi-asserted-by":"crossref","DOI":"10.1002\/cti2.1203","article-title":"Tocilizumab monotherapy uncovered the role of the CCL22\/17-CCR4+ Treg axis during remission of crescentic glomerulonephritis","volume":"9","author":"Sakai","year":"2020","journal-title":"Clin Transl Immunology"},{"key":"2025090306271928800_ref18","doi-asserted-by":"crossref","first-page":"2383","DOI":"10.1007\/s10067-017-3752-0","article-title":"Current clinical evidence of tocilizumab for the treatment of ANCA-associated vasculitis: a prospective case series for microscopic polyangiitis in a combination with corticosteroids and literature review","volume":"36","author":"Sakai","year":"2017","journal-title":"Clin Rheumatol"},{"key":"2025090306271928800_ref19","doi-asserted-by":"crossref","first-page":"900","DOI":"10.3109\/14397595.2016.1160968","article-title":"Corticosteroid-free treatment of tocilizumab monotherapy for microscopic polyangiitis: a single-arm, single-center, clinical trial","volume":"26","author":"Sakai","year":"2016","journal-title":"Mod Rheumatol"},{"key":"2025090306271928800_ref20","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1093\/mr\/roac114","article-title":"Systematic review and meta-analysis for 2023 clinical practice guidelines of the Japan Research Committee of the Ministry of Health, Labour, and Welfare for intractable vasculitis for the management of ANCA-associated vasculitis","volume":"33","author":"Watanabe","year":"2023","journal-title":"Mod Rheumatol"},{"key":"2025090306271928800_ref21","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1038\/s41577-023-00843-3","article-title":"Clonal haematopoiesis and dysregulation of the immune system","volume":"23","author":"Belizaire","year":"2023","journal-title":"Nat Rev Immunol"},{"key":"2025090306271928800_ref22","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1136\/ard-2023-224933","article-title":"Clonal haematopoiesis across the age spectrum of vasculitis patients with Takayasu's arteritis, ANCA-associated vasculitis and giant cell arteritis","volume":"83","author":"Gutierrez-Rodrigues","year":"2024","journal-title":"Ann Rheum Dis"},{"key":"2025090306271928800_ref23","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1681\/ASN.0000000000000116","article-title":"Transcriptional and clonal characterization of cytotoxic T cells in crescentic glomerulonephritis","volume":"34","author":"Mueller","year":"2023","journal-title":"J Am Soc Nephrol"},{"key":"2025090306271928800_ref24","doi-asserted-by":"crossref","first-page":"973","DOI":"10.1016\/j.kint.2023.01.023","article-title":"Identification of proteinase 3 autoreactive CD4(+)T cells and their T-cell receptor repertoires in antineutrophil cytoplasmic antibody-associated vasculitis","volume":"103","author":"Sharma","year":"2023","journal-title":"Kidney Int"},{"key":"2025090306271928800_ref25","first-page":"630","article-title":"Cytotoxic CX3CR1+ T cells drive vascular inflammation in giant cell arteritis but not in Takayasu's arteritis","volume":"43","author":"Inukai","year":"2025","journal-title":"Clin Exp Rheumatol"},{"key":"2025090306271928800_ref26","doi-asserted-by":"crossref","first-page":"621098","DOI":"10.3389\/fimmu.2020.621098","article-title":"Innate and adaptive immunity in giant cell arteritis","volume":"11","author":"Akiyama","year":"2021","journal-title":"Front Immunol"},{"key":"2025090306271928800_ref27","doi-asserted-by":"crossref","first-page":"2617","DOI":"10.3389\/fimmu.2019.02617","article-title":"Neutrophil extracellular traps induce tissue-invasive monocytes in granulomatosis with polyangiitis","volume":"10","author":"Akiyama","year":"2019","journal-title":"Front Immunol"}],"container-title":["Modern Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/mr\/advance-article-pdf\/doi\/10.1093\/mr\/roaf041\/63370661\/roaf041.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mr\/article-pdf\/35\/5\/878\/63370661\/roaf041.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/mr\/article-pdf\/35\/5\/878\/63370661\/roaf041.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T10:27:28Z","timestamp":1756895248000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/mr\/article\/35\/5\/878\/8151374"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5,27]]},"references-count":27,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2025,5,27]]},"published-print":{"date-parts":[[2025,9,2]]}},"URL":"https:\/\/doi.org\/10.1093\/mr\/roaf041","relation":{},"ISSN":["1439-7595","1439-7609"],"issn-type":[{"type":"print","value":"1439-7595"},{"type":"electronic","value":"1439-7609"}],"subject":[],"published-other":{"date-parts":[[2025,9]]},"published":{"date-parts":[[2025,5,27]]}}}