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Muscle-specific kinase-antibodies (MuSK-ABs) are detected in\u2009~\u20096% of MG, but data on outcome of MuSK-MCs are still lacking. We made a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody positive MG (AchR-MG) or MuSK-MG between 2006 and 2015 in a retrospective German multicenter study. We identified 19 MuSK-AB associated MCs in 15 patients and 161 MCs in 144 patients with AchR-ABs only. In contrast to patients with AchR-AB, MuSK-AB patients were more often female (<jats:italic>p<\/jats:italic>\u2009=\u20090.05, OR\u2009=\u20092.74) and classified as Myasthenia Gravis Foundation of America-class IV before crisis (<jats:italic>p<\/jats:italic>\u2009=\u20090.04, OR\u2009=\u20093.25). MuSK-AB patients suffer more often from multiple chronic disease (<jats:italic>p<\/jats:italic>\u2009=\u20090.016, OR\u2009=\u20094.87) and were treated more invasively in terms of plasma exchanging therapies (not significant). The number of days of mechanical ventilation (MV) (43.0\u2009\u00b1\u200953.1 vs. 17.4\u2009\u00b1\u200918; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001), days on an intensive care unit (ICU) (45.3\u2009\u00b1\u200949.5 vs. 21.2\u2009\u00b1\u200919.7; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001), and hospital-length of stay (LOS) (55.9\u2009\u00b1\u200947.6 vs. 28.8\u2009\u00b1\u200920.9 days; <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.0001) were significantly increased in MuSK-MC. Remarkable is that these changes were mainly due to patients with MusK-ABs only, whereas patients\u2019 outcome with both antibodies was similar to AchR-MCs. Furthermore, our data showed a shortened duration of MV after treatment with plasma exchanging therapies compared to treatment with intravenous immunoglobulin in MuSK-MCs. We conclude that MuSK-AB-status is associated with a longer need of MV, ICU-LOS, and hospital-LOS in MC, and therefore recommend early initiation of a disease-specific therapy.\n<\/jats:p>","DOI":"10.1007\/s00415-021-10603-9","type":"journal-article","created":{"date-parts":[[2021,5,10]],"date-time":"2021-05-10T08:03:48Z","timestamp":1620633828000},"page":"4824-4833","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation"],"prefix":"10.1007","volume":"268","author":[{"given":"Nicole","family":"K\u00f6nig","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henning 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Nicole K\u00f6nig reports no disclosures; Henning R. Stetefeld reports no disclosures; Christian Dohmen reports no disclosures; Philipp Mergenthaler is on the Advisory Board of HealthNextGen Inc. and has equity interest in the company; Siegfried Kohler reports no disclosures; Silvia Sch\u00f6nenberger reports no disclosures; Julian B\u00f6sel reports personal fees from Medtronic, personal fees from Zoll, personal fees from Boehringer Ingelheim, personal fees from Sedana Medical, and grants from PCORI, outside the submitted work; De-Hyung Lee reports no disclosures; Stefan T. Gerner reports no disclosures; Hagen B. Huttner reports no disclosures; Hauke Schneider reports no disclosures; Heinz Reichmann reports no disclosures; Hannah Fuhrer reports no disclosures; Benjamin Berger received travel grants and\/or training expenses from Bayer Vital GmbH, Ipsen Pharma GmbH, Novartis, Biogen GmbH, Merck Serono GmbH, and Genzyme, as well as lecture fees from Ipsen Pharma GmbH, Alexion Pharma Germany GmbH, Merck Serono GmbH, Sanofi Genzyme, and Roche Pharma AG outside the submitted work. Jan Zinke reports no disclosures; Anke Alberty reports no disclosures; Ingo Kleiter reports personal fees from Biogen, personal fees from Novartis, personal fees from Merck, personal fees from Sanofi Genzyme, personal fees from Roche, personal fees from Mylan, personal fees from Alexion, personal fees from Celgene, personal fees from Chugai, and personal fees from IQVIA, outside the submitted work; Christiane Schneider-Gold has received consulting and\/or speaker's honoraria from Alexion Pharmaceuticals, Amicus Therapeutics, Bayer Schering, CSL Behring, Gr\u00fcnenthal, Hormosan, Lupin Pharmaceuticals, and TEVA outside the submitted work. Christian Roth reports no disclosures; Juliane Dunkel reports no disclosures; Andreas Steinbrecher reports no disclosures; Andrea Thieme reports no disclosures; Felix Schlachetzki reports no disclosures related to the study and myasthenia gravis specifically; Ralf Linker reports no disclosures; Klemens Angstwurm reports no disclosures; Andreas Meisel reports personal fees from Alexion, personal fees from Grifols, grants from Octapharma, and personal fees from Hormosan, outside the submitted work; Bernhard Neumann reports no disclosures.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and\/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Local ethic committees and institutional review boards of the participating centers approved the study based on the central vote of the ethics committee at University of Regensburg (No: 15-101-0259). Due to the retrospective character of the study, patient\u2019s consent was not necessary according to the decisions of the ethic committees and institutional review boards.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}]}}