{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T18:21:35Z","timestamp":1782498095517,"version":"3.54.5"},"reference-count":50,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,10,17]],"date-time":"2025-10-17T00:00:00Z","timestamp":1760659200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>Primary angiitis of the CNS (PACNS) is a rare inflammatory disorder affecting blood vessels of the brain and spinal cord causing acute stroke. This study aimed to describe clinical, biochemical, imaging and histopathological findings of a retrospective single-center PACNS cohort in comparison to a cohort of secondary angiitis of CNS (SACNS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>All consecutive patients diagnosed with PACNS or SACNS between 2000 and 2023 were identified using our institutional database. Univariate comparison between both groups and multivariate analysis for independent predictors was performed, as well as Receiving Operating Characteristic analysis for white blood cell count predictive of PACNS. Kaplan\u2013Meier curves were used for evaluating survival outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified 20 patients in each group. PACNS patients presented more frequently with seizures (40% vs. 5%, <jats:italic>p<\/jats:italic>\u202f=\u202f0.02) and pseudotumoral lesions (45% vs. 10%, <jats:italic>p<\/jats:italic>\u202f=\u202f0.014). In PACNS patients, median serum WBC count at diagnosis was lower (8.4\u00d710<jats:sup>3<\/jats:sup>\/mm<jats:sup>3<\/jats:sup> [6.4\u20139.9] vs. 11.2\u00d710<jats:sup>3<\/jats:sup>\/mm<jats:sup>3<\/jats:sup> [9\u201313.6] <jats:italic>p<\/jats:italic>\u202f=\u202f0.027) and [18F]FDG-PET\/CT (<jats:italic>p<\/jats:italic>\u202f=\u202f0.001) was negative in all cases. No significant differences were observed for lumbar puncture profiles and diffusion weighted imaging patterns. Small-vessel vasculitis with a lymphocytic pattern was the most represented histologic phenotype in both groups. Serum WBC count \u22649.93 \u00d710<jats:sup>3<\/jats:sup>\/mm<jats:sup>3<\/jats:sup> was the only independent predictor of PACNS using multivariate analysis [OR 95%CI 5.107, (1.177\u201322.159)]. Both groups did not differ in terms of mortality, relapse and clinical outcome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In our study, PACNS patients presented more often with pseudotumoral lesions, seizures and small-vessel involvement and lymphocytic histologic pattern. WBC count \u22649.93\u00d710<jats:sup>3<\/jats:sup>\/mm<jats:sup>3<\/jats:sup> was an independent predictor of PACNS diagnosis.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fneur.2025.1602427","type":"journal-article","created":{"date-parts":[[2025,10,17]],"date-time":"2025-10-17T04:12:40Z","timestamp":1760674360000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Comparison between primary and secondary central nervous system vasculitis in terms of clinical, biochemical, radiological, histopathological features, and outcomes: a single-center retrospective cohort study"],"prefix":"10.3389","volume":"16","author":[{"given":"Pasquale","family":"Scoppettuolo","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucie","family":"Pothen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aline","family":"van Maanen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Onofrj","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Selda","family":"Aydin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Gheysens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renaud","family":"Lhommel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andr\u00e9","family":"Peeters","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincent","family":"van Pesch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Halil","family":"Yildiz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,10,17]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"603","DOI":"10.1016\/j.rdc.2023.03.011","article-title":"Central nervous system vasculitis: primary angiitis of the central nervous system and central nervous system manifestations of systemic vasculitis","volume":"49","author":"Amin","year":"2023","journal-title":"Rheum Dis Clin N Am"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"1028","DOI":"10.1056\/NEJMra2314942","article-title":"Primary central nervous system vasculitis","volume":"391","author":"Salvarani","year":"2024","journal-title":"N Engl J Med"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"442","DOI":"10.1002\/ana.21226","article-title":"Primary central nervous system vasculitis: analysis of 101 patients","volume":"62","author":"Salvarani","year":"2007","journal-title":"Ann Neurol"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"561","DOI":"10.1016\/S1474-4422(11)70081-3","article-title":"Primary angiitis of the CNS","volume":"10","author":"Hajj-Ali","year":"2011","journal-title":"Lancet Neurol"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1016\/j.jaut.2014.01.007","article-title":"Diagnosis and classification of central nervous system vasculitis","author":"Hajj-Ali","year":"2014","journal-title":"J Autoimmun"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1177\/1756285609347123","article-title":"Diagnosis and treatment of cerebral vasculitis","volume":"3","author":"Berlit","year":"2010","journal-title":"Ther Adv Neurol Disord"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1007\/s11926-018-0747-z","article-title":"CNS Vasculitis: an approach to differential diagnosis and management","volume":"20","author":"Byram","year":"2018","journal-title":"Curr Rheumatol Rep"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/art.37715","article-title":"2012 revised international Chapel Hill consensus conference nomenclature of vasculitides","volume":"65","author":"Jennette","year":"2013","journal-title":"Arthritis Rheum"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"749","DOI":"10.1136\/jnnp-2017-315691","article-title":"Subtypes of primary angiitis of the CNS identified by MRI patterns reflect the size of affected vessels","volume":"88","author":"Schuster","year":"2017","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"439","DOI":"10.1093\/rheumatology\/kew434","article-title":"Adult primary angiitis of the central nervous system: isolated small-vessel vasculitis represents distinct disease pattern","volume":"56","author":"Boysson","year":"2017","journal-title":"Rheumatol U K"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1097\/00005792-198801000-00002","article-title":"Primary angiitis of the central nervous system. 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