Konig, Nicole and Stetefeld, Henning R. and Dohmen, Christian and Mergenthaler, Philipp and Kohler, Siegfried and Schonenberger, Silvia and Bosel, Julian and Lee, De-Hyung and Gerner, Stefan T. and Huttner, Hagen B. and Schneider, Hauke and Reichmann, Heinz and Fuhrer, Hannah and Berger, Benjamin and Zinke, Jan and Alberty, Anke and Kleiter, Ingo and Schneider-Gold, Christiane and Roth, Christian and Dunkel, Juliane and Steinbrecher, Andreas and Thieme, Andrea and Schlachetzki, Felix and Linker, Ralf A. and Angstwurm, Klemens and Meisel, Andreas and Neumann, Bernhard (2021) MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation. JOURNAL OF NEUROLOGY, 268 (12). pp. 4824-4833. ISSN 0340-5354, 1432-1459
Full text not available from this repository. (Request a copy)Abstract
Myasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Muscle-specific kinase-antibodies (MuSK-ABs) are detected in similar to 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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | GRAVIS; AUTOANTIBODIES; MORTALITY; Myasthenia gravis; Myasthenic crisis; Autoimmune diseases; Antibody status; MuSK-antibodies; Outcome |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Neurologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 14 Sep 2022 05:35 |
| Last Modified: | 14 Sep 2022 05:35 |
| URI: | https://pred.uni-regensburg.de/id/eprint/47378 |
Actions (login required)
 |
View Item |
