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We aimed to independently validate the immunoassay for quantifying these antibodies and evaluate its diagnostic value for CAA\u2010RI.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We replicated the immunoassay to detect CSF anti\u2010A\u03b2 autoantibodies using CSF from CAA\u2010RI patients and non\u2010CAA controls with unrelated disorders and further characterized its performance. Moreover, we conducted a literature review of CAA\u2010RI case reports to investigate neuropathological and CSF evidence of the nature of the inflammatory reaction in CAA\u2010RI.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The assay demonstrated a high background signal in CSF, which increased and corresponded with higher total immunoglobulin G (IgG) concentration in CSF (<jats:italic>r<\/jats:italic><jats:sub>sp<\/jats:sub>\u2009=\u20090.51, <jats:italic>p<\/jats:italic>\u2009=\u20090.02). Assay levels were not elevated in CAA\u2010RI patients (<jats:italic>n<\/jats:italic>\u2009=\u20096) compared to non\u2010CAA controls (<jats:italic>n<\/jats:italic>\u2009=\u200920; <jats:italic>p<\/jats:italic>\u2009=\u20090.64). Literature review indicated only occasional presence of B\u2010lymphocytes and plasma cells (i.e., antibody\u2010producing cells), alongside the abundant presence of activated microglial cells, T\u2010cells, and other monocyte lineage cells. CSF analysis did not convincingly indicate intrathecal IgG production.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>We were unable to reproduce the reported elevation of anti\u2010A\u03b2 autoantibody concentration in CSF of CAA\u2010RI patients. Our findings instead support nonspecific detection of IgG levels in CSF by the assay. Reviewed CAA\u2010RI case reports suggested a widespread cerebral inflammatory reaction. In conclusion, our findings do not support anti\u2010A\u03b2 autoantibodies as a diagnostic biomarker for CAA\u2010RI.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.52169","type":"journal-article","created":{"date-parts":[[2024,9,13]],"date-time":"2024-09-13T13:02:56Z","timestamp":1726232576000},"page":"2563-2571","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["No replicating evidence for anti\u2010amyloid\u2010\u03b2 autoantibodies in cerebral amyloid angiopathy\u2010related inflammation"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9004-934X","authenticated-orcid":false,"given":"Emma","family":"van den Berg","sequence":"first","affiliation":[{"name":"Department of Neurology, Donders Institute for Brain, Cognition and Behaviour Radboud University Medical Center  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rian","family":"Roelofs","sequence":"additional","affiliation":[{"name":"Department of Human Genetics Radboud University Medical Center  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lieke","family":"J\u00e4kel","sequence":"additional","affiliation":[{"name":"Department of Neurology, Donders Institute for Brain, Cognition and Behaviour Radboud University Medical Center  Nijmegen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steven M.","family":"Greenberg","sequence":"additional","affiliation":[{"name":"Department of Neurology Massachusetts General Hospital  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Charidimou","sequence":"additional","affiliation":[{"name":"Department of Neurology, Boston University Medical Center Boston University Chobanian &amp; Avedisian School of Medicine  Boston Massachusetts USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8301-6846","authenticated-orcid":false,"given":"Ellis S.","family":"van Etten","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center  Leiden the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Delphine","family":"Boche","sequence":"additional","affiliation":[{"name":"Clinical Neurosciences, Clinical and Experimental Sciences, Faculty of Medicine University of Southampton  Southampton UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8495-4578","authenticated-orcid":false,"given":"Catharina J. 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