{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,11]],"date-time":"2026-10-11T14:31:19Z","timestamp":1791729079047,"version":"4.3.4"},"reference-count":28,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2023,2,24]],"date-time":"2023-02-24T00:00:00Z","timestamp":1677196800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["TR\u2010128"],"award-info":[{"award-number":["TR\u2010128"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2023,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Complement component 5 (C5) targeting therapies are clinically beneficial in patients with acetylcholine receptor antibody<jats:sup>+<\/jats:sup> (AChR\u2010Ab<jats:sup>+<\/jats:sup>) generalized myasthenia gravis (MG). That clearly implicates antibody\u2010mediated complement activation in MG pathogenesis. Here, classical and alternative complement pathways were profiled in patients from different MG subgroups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In a case\u2013control study, concentrations of C3a, C5a and sC5b9 were simultaneously quantified, indicating general activation of the complement system, whether via the classical and lectin pathways (C4a) or the alternative pathway (factors Ba and Bb) in MG patients with AChR or muscle\u2010specific kinase antibodies (MuSK\u2010Abs) or seronegative MG compared to healthy donors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Treatment\u2010na\u00efve patients with AChR\u2010Ab<jats:sup>+<\/jats:sup> MG showed substantially increased plasma levels of cleaved complement components, indicating activation of the classical and alternative as well as the terminal complement pathways. These increases were still present in a validation cohort of AChR\u2010Ab<jats:sup>+<\/jats:sup> patients under standard immunosuppressive therapies; notably, they were not evident in patients with MuSK\u2010Abs or seronegative MG. Neither clinical severity parameters (at the time of sampling or 1\u2009year later) nor anti\u2010AChR titres correlated significantly with activated complement levels.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Markers indicative of complement activation are prominently increased in patients with AChR\u2010Ab MG despite standard immunosuppressive therapies. Complement inhibition proximal to C5 cleavage should be explored for its potential therapeutic benefits in AChR\u2010Ab<jats:sup>+<\/jats:sup> MG.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.15730","type":"journal-article","created":{"date-parts":[[2023,2,8]],"date-time":"2023-02-08T09:00:32Z","timestamp":1675846832000},"page":"1409-1416","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Complement activation profiles in <scp>anti\u2010acetylcholine receptor<\/scp> positive myasthenia gravis"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5306-7880","authenticated-orcid":false,"given":"Frauke","family":"Stascheit","sequence":"first","affiliation":[{"name":"Department of Neurology with Experimental Neurology Charit\u00e9\u2014Universit\u00e4tsmedizin Berlin  Berlin Germany"},{"name":"NeuroCure Clinical Research Center Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate 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