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This study aimed to determine the frequency of CVEs at the time of diagnosis of PSV, to identify factors associated with CVEs in PSV, and to explore features and outcomes of stroke in patients with PSV.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Data from adults newly diagnosed with PSV within the Diagnostic and Classification Criteria in VASculitis (DCVAS) study were analysed. Demographics, risk factors for vascular disease, and clinical features were compared between patients with PSV with and without CVE. Stroke subtypes and cumulative incidence of recurrent CVE during a prospective 6-month follow-up were also assessed.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>The analysis included 4828 PSV patients, and a CVE was reported in 169 (3.50%, 95% CI 3.00\u20134.06): 102 (2.13% 95% CI 1.73\u20132.56) with stroke and 81 (1.68% 95% CI 1.33\u20132.08) with transient ischemic attack (TIA). The frequency of CVE was highest in Beh\u00e7et\u2019s disease (9.5%, 95% CI 5.79\u201314.37), polyarteritis nodosa (6.2%, 95% CI 3.25\u201310.61), and Takayasu\u2019s arteritis (6.0%, 95% CI 4.30\u20138.19), and lowest in microscopic polyangiitis (2.2%, 95% CI 1.09\u20133.86), granulomatosis with polyangiitis (2.0%, 95% CI 1.20\u20133.01), cryoglobulinaemic vasculitis (1.9%, 95% CI 0.05\u20139.89), and IgA-vasculitis (Henoch-Sch\u00f6nlein) (0.4%, 95% CI 0.01\u20132.05). PSV patients had a 11.9% cumulative incidence of recurrent CVE during a 6-month follow-up period.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>CVEs affect a significant proportion of patients at time of PSV diagnosis, and the frequency varies widely among different vasculitis, being higher in Beh\u00e7et\u2019s. Overall, CVE in PSV is not explained by traditional vascular risk factors and has a high risk of CVE recurrence.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00415-024-12251-1","type":"journal-article","created":{"date-parts":[[2024,3,12]],"date-time":"2024-03-12T23:02:35Z","timestamp":1710284555000},"page":"3309-3320","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Stroke frequency, associated factors, and clinical features in primary systemic vasculitis: a multicentric observational study"],"prefix":"10.1007","volume":"271","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5829-808X","authenticated-orcid":false,"given":"Ruth","family":"Geraldes","sequence":"first","affiliation":[]},{"given":"Monica","family":"Santos","sequence":"additional","affiliation":[]},{"given":"Cristina","family":"Ponte","sequence":"additional","affiliation":[]},{"given":"Anthea","family":"Craven","sequence":"additional","affiliation":[]},{"given":"Lillian","family":"Barra","sequence":"additional","affiliation":[]},{"given":"Joanna C.","family":"Robson","sequence":"additional","affiliation":[]},{"given":"Nevin","family":"Hammam","sequence":"additional","affiliation":[]},{"given":"Jason","family":"Springer","sequence":"additional","affiliation":[]},{"given":"J\u00f6erg","family":"Henes","sequence":"additional","affiliation":[]},{"given":"Alojzija","family":"Hocevar","sequence":"additional","affiliation":[]},{"given":"Jukka","family":"Putaala","sequence":"additional","affiliation":[]},{"given":"Ernestina","family":"Santos","sequence":"additional","affiliation":[]},{"given":"Liza","family":"Rajasekhar","sequence":"additional","affiliation":[]},{"given":"Thomas","family":"Daikeler","sequence":"additional","affiliation":[]},{"given":"Omer","family":"Karadag","sequence":"additional","affiliation":[]},{"given":"Andreia","family":"Costa","sequence":"additional","affiliation":[]},{"given":"Nader","family":"Khalidi","sequence":"additional","affiliation":[]},{"given":"Christian","family":"Pagnoux","sequence":"additional","affiliation":[]},{"given":"Patr\u00edcia","family":"Canh\u00e3o","sequence":"additional","affiliation":[]},{"given":"Teresa Pinho e","family":"Melo","sequence":"additional","affiliation":[]},{"given":"Ana Catarina","family":"Fonseca","sequence":"additional","affiliation":[]},{"given":"Jos\u00e9 M.","family":"Ferro","sequence":"additional","affiliation":[]},{"given":"Jo\u00e3o Eurico","family":"Fonseca","sequence":"additional","affiliation":[]},{"given":"Ravi","family":"Suppiah","sequence":"additional","affiliation":[]},{"given":"Richard A.","family":"Watts","sequence":"additional","affiliation":[]},{"given":"Peter","family":"Grayson","sequence":"additional","affiliation":[]},{"given":"Peter A.","family":"Merkel","sequence":"additional","affiliation":[]},{"given":"Raashid A.","family":"Luqmani","sequence":"additional","affiliation":[]},{"name":"the DCVAS Study Group","sequence":"additional","affiliation":[]}],"member":"297","published-online":{"date-parts":[[2024,3,12]]},"reference":[{"key":"12251_CR1","doi-asserted-by":"publisher","first-page":"411","DOI":"10.1136\/jnnp-2019-322424","volume":"91","author":"E Boot","year":"2020","unstructured":"Boot E, Ekker MS, Putaala J et al (2020) Ischaemic stroke in young adults: a global perspective. 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M Santos reports no disclosures relevant to the manuscript. C Ponte reports no disclosures relevant to the manuscript. A Craven reports no disclosures relevant to the manuscript. L Barra reports no disclosures relevant to the manuscript. JC Robson reports no disclosures relevant to the manuscript. N Hammam reports no disclosures relevant to the manuscript. J Springer no disclosures relevant to the manuscript. J Henes reports no disclosures relevant to the manuscript. A Hocevar reports no disclosures relevant to the manuscript. J Putaala reports no disclosures relevant to the manuscript. E Santos reports no disclosures relevant to the manuscript. L Rajasekhar reports no disclosures relevant to the manuscript. T Daikeler reports no disclosures relevant to the manuscript. O Karadag reports no disclosures relevant to the manuscript. A Costa reports no disclosures relevant to the manuscript. N Khalidi reports no disclosures relevant to the manuscript. C Pagnoux reports no disclosures relevant to the manuscript. P Canh\u00e3o reports no disclosures relevant to the manuscript Teresa. P. Melo reports no disclosures relevant to the manuscript A C. Fonseca reports no disclosures relevant to the manuscript. J M. Ferro reports no disclosures relevant to the manuscript. J E. Fonseca reports no disclosures relevant to the manuscript. R Suppiah reports no disclosures relevant to the manuscript. R A. Watts reports no disclosures relevant to the manuscript. P Grayson reports no disclosures relevant to the manuscript. P A. Merkel reports no disclosures relevant to the manuscript. R A. Luqmani reports no disclosures relevant to the manuscript. R Watts disclosures: Editor in Chief Oxford Medical Case Reports\u2014since 2012; Data monitoring committees; Chair DMEC for the BIOVASS trial funded by NIHR; Chair trial steering committee Combivas trial funded MRC and GSK; Royalties Oxford Textbook of Rheumatology Editor 4th edition published 2014.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"All human and animal studies have been approved by the appropriate ethics committee and have therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki\u00a0and its later amendments. It should also be stated clearly in the text that all persons gave their informed consent prior to their inclusion in the study.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics"}}]}}