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

  1. König, Nicole
  2. Stetefeld, Henning R.
  3. Dohmen, Christian
  4. Mergenthaler, Philipp
  5. Kohler, Siegfried
  6. Schönenberger, Silvia
  7. Bösel, Julian
  8. Lee, De-Hyung
  9. Gerner, Stefan T.
  10. Huttner, Hagen B.
  11. Schneider, Hauke
  12. Reichmann, Heinz
  13. Fuhrer, Hannah
  14. Berger, Benjamin
  15. Zinke, Jan
  16. Alberty, Anke
  17. Kleiter, Ingo
  18. Schneider-Gold, Christiane
  19. Roth, Christian
  20. Dunkel, Juliane
  21. Steinbrecher, Andreas
  22. Thieme, Andrea
  23. Schlachetzki, Felix
  24. Linker, Ralf A.
  25. Angstwurm, Klemens
  26. Meisel, Andreas
  27. Neumann, Bernhard ORCID logo
  28. Fuchs, Kornelius
  29. Schalke, Berthold
  30. Vidal, Amelie
  31. Brachaczek, Izabela
  32. Maidhof, Jana
  33. Wenke, Arno
  34. Hagen, Manuel
  35. Rahmig, Jan
  36. Schimmel, Eik
  37. Niesen, Wolf
  38. Fahrendorf, Christine

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WeightNameValue
1000 Titel
  • MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation
1000 Autor/in
  1. König, Nicole |
  2. Stetefeld, Henning R. |
  3. Dohmen, Christian |
  4. Mergenthaler, Philipp |
  5. Kohler, Siegfried |
  6. Schönenberger, Silvia |
  7. Bösel, Julian |
  8. Lee, De-Hyung |
  9. Gerner, Stefan T. |
  10. Huttner, Hagen B. |
  11. Schneider, Hauke |
  12. Reichmann, Heinz |
  13. Fuhrer, Hannah |
  14. Berger, Benjamin |
  15. Zinke, Jan |
  16. Alberty, Anke |
  17. Kleiter, Ingo |
  18. Schneider-Gold, Christiane |
  19. Roth, Christian |
  20. Dunkel, Juliane |
  21. Steinbrecher, Andreas |
  22. Thieme, Andrea |
  23. Schlachetzki, Felix |
  24. Linker, Ralf A. |
  25. Angstwurm, Klemens |
  26. Meisel, Andreas |
  27. Neumann, Bernhard |
  28. Fuchs, Kornelius |
  29. Schalke, Berthold |
  30. Vidal, Amelie |
  31. Brachaczek, Izabela |
  32. Maidhof, Jana |
  33. Wenke, Arno |
  34. Hagen, Manuel |
  35. Rahmig, Jan |
  36. Schimmel, Eik |
  37. Niesen, Wolf |
  38. Fahrendorf, Christine |
1000 Erscheinungsjahr 2021
1000 Publikationstyp
  1. Artikel |
1000 Online veröffentlicht
  • 2021-05-10
1000 Erschienen in
1000 Quellenangabe
  • 268(12):4824-4833
1000 Copyrightjahr
  • 2021
1000 Lizenz
1000 Verlagsversion
  • https://doi.org/10.1007/s00415-021-10603-9 |
  • https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8563593/ |
1000 Publikationsstatus
1000 Begutachtungsstatus
1000 Sprache der Publikation
1000 Abstract/Summary
  • 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.
1000 Sacherschließung
lokal Antibody status
lokal Female [MeSH]
lokal Outcome
lokal MuSK-antibodies
lokal Myasthenia Gravis/therapy [MeSH]
lokal Autoimmune diseases
lokal Humans [MeSH]
lokal Retrospective Studies [MeSH]
lokal Myasthenic crisis
lokal Myasthenia gravis
lokal Antibodies/blood [MeSH]
lokal Receptors, Cholinergic/immunology [MeSH]
lokal Autoantibodies [MeSH]
lokal Respiration, Artificial [MeSH]
lokal Myasthenia Gravis/complications [MeSH]
lokal Original Communication
lokal Receptor Protein-Tyrosine Kinases/immunology [MeSH]
1000 Liste der Beteiligten
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1000 Erstellt am 2023-05-09T11:45:59.826+0200
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