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Although reported in a few studies, the prevalence of large vessel vasculitis (LVV) in patients with AAV remains to be further explored. The goal of the present study was\u00a0to assess the prevalence of LVV in a cohort of patients with AAV and to characterize this population. We conducted a\u00a0ten-year retrospective study of a single-center cohort of AAV, including\u00a0101 patients with GPA (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200958), EGPA (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200928), MPA (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200915), and\u00a0compared the\u00a0groups with or without associated LVV. LVV was diagnosed in five patients, two with aortitis and three with temporal arteritis, corresponding to a total prevalence of 5.0% [95% CI 1.6\u201311.2%]. This value was significantly higher than the estimated prevalence of LVV in the normal Swiss population (OR 234.9 95% CI 91.18\u2013605.2,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001). All five patients had GPA, whereas no cases with EGPA or MPA\u00a0were identified. Anti-PR3 antibodies were detected in four out of five patients, anti-MPO in one patient. Since LVV can occur in a significant proportion of patients with GPA, evaluation for LVV may be considered systematically\u00a0in the diagnostic workup\u00a0of AAV.\n                  <\/jats:p>","DOI":"10.1007\/s00296-021-04993-2","type":"journal-article","created":{"date-parts":[[2021,9,24]],"date-time":"2021-09-24T16:09:14Z","timestamp":1632499754000},"page":"2147-2156","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Prevalence of large vessel vasculitis in ANCA-associated vasculitis: a retrospective cohort study"],"prefix":"10.1007","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9697-7526","authenticated-orcid":false,"given":"Yann","family":"Coattrenec","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0513-7156","authenticated-orcid":false,"given":"Yannick D.","family":"Muller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2575-9537","authenticated-orcid":false,"given":"David","family":"Spoerl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6963-9342","authenticated-orcid":false,"given":"Johannes A.","family":"Lobrinus","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5748-4577","authenticated-orcid":false,"given":"J\u00f6rg D.","family":"Seebach","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,9,24]]},"reference":[{"issue":"1","key":"4993_CR1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/Art.37715","volume":"65","author":"J Jc","year":"2013","unstructured":"Jc J, Rj F, Pa B et al (2013) 2012 revised international chapel hill consensus conference nomenclature of vasculitides. 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