Universität zu Köln

Nelke, Christopher, Stascheit, Frauke ORCID: 0000-0001-5306-7880, Eckert, Carmen, Pawlitzki, Marc, Schroeter, Christina B., Huntemann, Niklas, Mergenthaler, Philipp, Arat, Ercan, ozturk, Menekse, Foell, Dirk, Schreiber, Stefanie, Vielhaber, Stefan, Gassa, Asmae, Stetefeld, Henning, Schroeter, Michael, Berger, Benjamin ORCID: 0000-0003-2654-2898, Totzeck, Andreas, Hagenacker, Tim, Meuth, Sven G., Meisel, Andreas ORCID: 0000-0001-7233-5342, Wiendl, Heinz and Ruck, Tobias (2022). Independent risk factors for myasthenic crisis and disease exacerbation in a retrospective cohort of myasthenia gravis patients. J. Neuroinflamm., 19 (1). LONDON: BMC. ISSN 1742-2094

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Abstract

Background: Myasthenic crisis (MC) and disease exacerbation in myasthenia gravis (MG) are associated with significant lethality and continue to impose a high disease burden on affected patients. Therefore, we sought to determine potential predictors for MC and exacerbation as well as to identify factors affecting outcome. Methods: We examined a retrospective, observational cohort study of patients diagnosed with MG between 2000 and 2021 with a mean follow-up of 62.6 months after diagnosis from eight tertiary hospitals in Germany. A multivariate Cox regression model with follow-up duration as the time variable was used to determine independent risk factors for MC and disease exacerbation. Results: 815 patients diagnosed with MG according to national guidelines were included. Disease severity at diagnosis (quantitative MG score or Myasthenia Gravis Foundation of America class), the presence of thymoma and antimuscle specific tyrosine kinase-antibodies were independent predictors of MC or disease exacerbation. Patients with minimal manifestation status 12 months after diagnosis had a lower risk of MC and disease exacerbation than those without. The timespan between diagnosis and the start of immunosuppressive therapy did not affect risk. Patients with a worse outcome of MC were older, had higher MGFA class before MC and at admission, and had lower vital capacity before and at admission. The number of comorbidities, requirement for intubation, prolonged mechanical ventilation, and MC triggered by infection were associated with worse outcome. No differences between outcomes were observed comparing treatments with IVIG (intravenous immunoglobulin) vs. plasma exchange vs. IVIG together with plasma exchange. Conclusions: MC and disease exacerbations inflict a substantial burden of disease on MG patients. Disease severity at diagnosis and antibody status predicted the occurrence of MC and disease exacerbation. Intensified monitoring with emphasis on the prevention of infectious complications could be of value to prevent uncontrolled disease in MG patients.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Nelke, Christopher UNSPECIFIED UNSPECIFIED UNSPECIFIED Stascheit, Frauke UNSPECIFIED orcid.org/0000-0001-5306-7880 UNSPECIFIED Eckert, Carmen UNSPECIFIED UNSPECIFIED UNSPECIFIED Pawlitzki, Marc UNSPECIFIED UNSPECIFIED UNSPECIFIED Schroeter, Christina B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Huntemann, Niklas UNSPECIFIED UNSPECIFIED UNSPECIFIED Mergenthaler, Philipp UNSPECIFIED UNSPECIFIED UNSPECIFIED Arat, Ercan UNSPECIFIED UNSPECIFIED UNSPECIFIED ozturk, Menekse UNSPECIFIED UNSPECIFIED UNSPECIFIED Foell, Dirk UNSPECIFIED UNSPECIFIED UNSPECIFIED Schreiber, Stefanie UNSPECIFIED UNSPECIFIED UNSPECIFIED Vielhaber, Stefan UNSPECIFIED UNSPECIFIED UNSPECIFIED Gassa, Asmae UNSPECIFIED UNSPECIFIED UNSPECIFIED Stetefeld, Henning UNSPECIFIED UNSPECIFIED UNSPECIFIED Schroeter, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Berger, Benjamin UNSPECIFIED orcid.org/0000-0003-2654-2898 UNSPECIFIED Totzeck, Andreas UNSPECIFIED UNSPECIFIED UNSPECIFIED Hagenacker, Tim UNSPECIFIED UNSPECIFIED UNSPECIFIED Meuth, Sven G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Meisel, Andreas UNSPECIFIED orcid.org/0000-0001-7233-5342 UNSPECIFIED Wiendl, Heinz UNSPECIFIED UNSPECIFIED UNSPECIFIED Ruck, Tobias UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-693036
DOI:	10.1186/s12974-022-02448-4
Journal or Publication Title:	J. Neuroinflamm.
Volume:	19
Number:	1
Date:	2022
Publisher:	BMC
Place of Publication:	LONDON
ISSN:	1742-2094
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language INTERNATIONAL CONSENSUS GUIDANCE; MECHANICAL VENTILATION; MANAGEMENT; ANTIBODIES; MORTALITY; TRIAL Multiple languages Immunology; Neurosciences Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/69303