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MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation

Konig N, Stetefeld HR, Dohmen C, Mergenthaler P, Kohler S, Schonenberger S, Bosel J, Lee DH, Gerner S, Huttner H, Schneider H, Reichmann H, Fuhrer H, Berger B, Zinke J, Alberty A, Kleiter I, Schneider-Gold C, Roth C, Dunkel J, Steinbrecher A, Thieme A, Schlachetzki F, Linker RA, Angstwurm K, Meisel A, Neumann B (2021)

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Publication Type: Journal article

Publication year: 2021

Journal

DOI: 10.1007/s00415-021-10603-9

Abstract

Myasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Muscle-specific kinase-antibodies (MuSK-ABs) are detected in ~ 6% 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 (p = 0.05, OR = 2.74) and classified as Myasthenia Gravis Foundation of America-class IV before crisis (p = 0.04, OR = 3.25). MuSK-AB patients suffer more often from multiple chronic disease (p = 0.016, OR = 4.87) and were treated more invasively in terms of plasma exchanging therapies (not significant). The number of days of mechanical ventilation (MV) (43.0 ± 53.1 vs. 17.4 ± 18; p < 0.0001), days on an intensive care unit (ICU) (45.3 ± 49.5 vs. 21.2 ± 19.7; p < 0.0001), and hospital-length of stay (LOS) (55.9 ± 47.6 vs. 28.8 ± 20.9 days; p < 0.0001) were significantly increased in MuSK-MC. Remarkable is that these changes were mainly due to patients with MusK-ABs only, whereas patients’ 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.

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APA:

Konig, N., Stetefeld, H.R., Dohmen, C., Mergenthaler, P., Kohler, S., Schonenberger, S.,... Neumann, B. (2021). MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation. Journal of Neurology. https://doi.org/10.1007/s00415-021-10603-9

MLA:

Konig, Nicole, et al. "MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation." Journal of Neurology (2021).

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