{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T23:13:47Z","timestamp":1777936427553,"version":"3.51.4"},"reference-count":48,"publisher":"SAGE Publications","issue":"9","license":[{"start":{"date-parts":[[2011,5,4]],"date-time":"2011-05-04T00:00:00Z","timestamp":1304467200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Mult Scler"],"published-print":{"date-parts":[[2011,9]]},"abstract":"<jats:p>Background: A new autoantibody (termed NMO-IgG, or AQP4-Ab) has recently been described in patients with neuromyelitis optica (NMO) and its formes frustes, longitudinally extensive transverse myelitis (LETM) and recurrent optic neuritis (rON). However, AQP4-Ab has been found also in patients with co-existing rheumatic diseases such as systemic lupus erythematosus (SLE) or Sj\u00f6gren\u2019s syndrome (SS), conditions which are characterized by broad, polyspecific B cell activation.<\/jats:p>\n                  <jats:p>Objectives: In this study, we aimed at evaluating the syndrome specificity and frequency of AQP4-Ab in patients with rheumatic diseases and neurological symptoms.<\/jats:p>\n                  <jats:p>Methods: For this purpose, serum samples from 109 neurological patients with established connective tissue disorders (CTD) ( n = 54), possible CTD ( n = 42), or vasculitis ( n = 13) were analysed for the presence of AQP4-Ab by a cell-based assay employing recombinant human AQP4.<\/jats:p>\n                  <jats:p>Results: AQP4-Ab was detectable in 31\/40 (78%) patients with CTD and NMO spectrum disorders (median titre, 1:1000) but in none of the samples obtained from patients with CTD or vasculitis and neurological disorders other than NMO, LETM, or rON ( n = 69).<\/jats:p>\n                  <jats:p>Conclusion: The high syndrome specificity of the antibody for neuromyelitis optica spectrum disorders (NMOSDs) in patients with CTD supports the concept of AQP4-Ab being involved in the pathogenesis of these neurological conditions, and argues against AQP4-Ab simply being part of the polyclonal B cell activation generally associated with rheumatic diseases. Moreover, the finding that AQP4-Ab is present in patients with CTD and co-existing NMOSD with approximately the same frequency as in patients without CTD strengthens the case of CTD and AQP4-Ab positive NMOSD representing two co-existing yet distinct entities in the majority of patients.<\/jats:p>","DOI":"10.1177\/1352458511403958","type":"journal-article","created":{"date-parts":[[2011,5,4]],"date-time":"2011-05-04T23:51:03Z","timestamp":1304553063000},"page":"1067-1073","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":138,"title":["Frequency and syndrome specificity of antibodies to aquaporin-4 in neurological patients with rheumatic disorders"],"prefix":"10.1177","volume":"17","author":[{"given":"Sven","family":"Jarius","sequence":"first","affiliation":[{"name":"Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"Jacobi","sequence":"additional","affiliation":[{"name":"Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jerome","family":"de Seze","sequence":"additional","affiliation":[{"name":"Clinique Neurologique, CHU de Strasbourg, Strasbourg, France."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Helene","family":"Zephir","sequence":"additional","affiliation":[{"name":"P\u00f4le Neurologique, H\u00f4pital Roger Salengro, CHRU de Lille, Lille, France."},{"name":"Laboratoire d\u2019Immunologie, Universit\u00e9 Lille Nord de France, Lille, France."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Friedemann","family":"Paul","sequence":"additional","affiliation":[{"name":"NeuroCure Clinical Research Center, Charit\u00e9 \u2013 University Medicine Berlin, Berlin, Germany."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Diego","family":"Franciotta","sequence":"additional","affiliation":[{"name":"IRCCS, National Neurological Institute \u2018C. Mondino\u2019, Pavia, Italy."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paulus","family":"Rommer","sequence":"additional","affiliation":[{"name":"Department of Neurology, Medical University of Vienna, Vienna, Austria."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Simone","family":"Mader","sequence":"additional","affiliation":[{"name":"Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, Austria."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ingo","family":"Kleiter","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Medical Centre Regensburg, Regensburg, Germany."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Markus","family":"Reindl","sequence":"additional","affiliation":[{"name":"Clinical Department of Neurology, Innsbruck Medical University, Innsbruck, Austria."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gulsen","family":"Akman-Demir","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Istanbul, Istanbul, Turkey."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Seifert-Held","sequence":"additional","affiliation":[{"name":"Department of Neurology, Graz Medical University, Graz, Austria."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"Kristoferitsch","sequence":"additional","affiliation":[{"name":"Department of Neurology, Sozialmedizinisches Zentrum \u2013 Donauspital, Vienna, Austria."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arthur","family":"Melms","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Tuebingen, Tuebingen, Germany."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Klaus-Peter","family":"Wandinger","sequence":"additional","affiliation":[{"name":"Institute for Experimental Immunology, affiliated to Euroimmun, Luebeck, Germany."},{"name":"Institute for Neuroimmunology and Clinical MS Research, University Medical Center Eppendorf, Hamburg, Germany."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brigitte","family":"Wildemann","sequence":"additional","affiliation":[{"name":"Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Heidelberg, Germany."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2011,5,4]]},"reference":[{"key":"bibr1-1352458511403958","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000216139.44259.74"},{"key":"bibr2-1352458511403958","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20770"},{"key":"bibr3-1352458511403958","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000303817.82134.da"},{"key":"bibr4-1352458511403958","doi-asserted-by":"crossref","unstructured":"Jarius S, Frederikson J, Waters P, Paul F, Akman-Demir G, Marignier R, et al. Frequency and prognostic impact of antibodies to aquaporin-4 in patients with optic neuritis.\n                      J Neurol Sci\n                      2010; 298(1\u20132): 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