{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T06:09:30Z","timestamp":1778479770976,"version":"3.51.4"},"reference-count":19,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T00:00:00Z","timestamp":1778457600000},"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. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Autoinflammatory diseases (AIDs) are characterized by recurrent episodes of inflammation caused by primary immune system dysregulation. Central nervous manifestations, including aseptic meningitis (AM), are rare in AIDs. In this study, we identified six patients with undetermined AIDs characterized by recurrent aseptic meningitis (RAM).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients referred to Centre National de R\u00e9f\u00e9rence des Maladies Auto-Inflammatoires for AM with an undetermined diagnosis were included. AIDs with RAM were defined by two or more episodes of aseptic meningitis (&amp;gt;10 leukocytes) with a predominance of neutrophils (&amp;gt;50%) and an association with autoinflammatory elements (i.e., fever, C-reactive protein [CRP] elevation) and the absence of an alternative diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Six patients (four men, two women) were included, all of whom presented with fever and elevated CRP levels (\u226530 mg\/L), and five patients had clinical meningitis syndrome. The median patient age at disease onset was 41 years (range, 28\u201349). The frequency of AM was 1\u201324 episodes\/year, and the duration of episodes was \u22648 days. Associated clinical symptoms included cutaneous manifestations (rash [n = 3], Raynaud\u2019s syndrome [n = 1]), arthromyalgia (n = 2), and central neurological or ophthalmologic manifestations (n = 2). All patients underwent genetic analysis focusing on AIDs, and variants of undetermined significance within four autoinflammatory genes were noted in three patients. All patients benefited from classical therapeutic regimens of AIDs including corticosteroids, colchicine, and anti-interleukin (IL)-1 receptor or anti-IL6 antibodies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>RAM was identified as a potential indicator for undetermined AIDs. Physicians should be aware of this condition and provide adequate analysis and treatment.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2026.1842256","type":"journal-article","created":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T05:30:28Z","timestamp":1778477428000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Recurrent aseptic meningitis in adults: a potential indicator of undetermined autoinflammatory disease"],"prefix":"10.3389","volume":"17","author":[{"given":"Martin","family":"Martinot","sequence":"first","affiliation":[{"name":"Service de Maladies Infectieuse, H\u00f4pitaux Civils de Colmar","place":["Colmar, France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samuel","family":"Ardois","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, CHRU Rennes","place":["Rennes, France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elodie","family":"Chauvet","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, CHRU Montpellier","place":["Montpellier, France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Cavestro","sequence":"additional","affiliation":[{"name":"Service de Maladies Infectieuse, H\u00f4pitaux Civils de Colmar","place":["Colmar, France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philippe","family":"Blanche","sequence":"additional","affiliation":[{"name":"D\u00e9partement de M\u00e9decine Interne, H\u00f4pital Cochin, APHP","place":["Paris, France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Papo","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, H\u00f4pital Bichat, Universit\u00e9 Paris Cit\u00e9","place":["Paris, France"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne","family":"Murarasu","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, CHRU Tours","place":["Tours, France"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,5,11]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurol.2019.07.005","article-title":"Aseptic meningitis","volume":"175","author":"Tattevin","year":"2019","journal-title":"Rev Neurol (Paris)"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1097\/QCO.0000000000001105","article-title":"Aseptic meningitis: a foundation review","volume":"38","author":"Allos","year":"2025","journal-title":"Curr Opin Infect Dis"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2017-212515","article-title":"Consensus proposal for taxonomy and definition of the autoinflammatory diseases (AIDs): a Delphi study","volume":"77","author":"Ben-Chetrit","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-018-4209-9","article-title":"From autoinflammation to autoimmunity: old and recent findings","volume":"37","author":"Caso","year":"2018","journal-title":"Clin Rheumatol"},{"key":"B5","article-title":"Neurological manifestations in autoinflammatory diseases","author":"Uccelli","year":"2018","journal-title":"Clin Exp Rheumatol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2019.12.003","article-title":"Neurological manifestations of autoinflammatory diseases in Chinese adult patients","volume":"50","author":"Qin","year":"2020","journal-title":"Semin Arthritis Rheum"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1111\/jdv.18771","article-title":"Systemic inflammatory trunk recurrent acute macular eruption (SITRAME): a new auto-inflammatory syndrome in adult","volume":"37","author":"Soria","year":"2023","journal-title":"J Eur Acad Dermatol Venereol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1016\/j.revmed.2025.11.003","article-title":"What is an undifferentiated systemic autoinflammatory disease in adults? 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