Abstract
Objective
This study aimed to investigate the timing of meeting the criteria for a status of “minimal manifestation (MM) or better” and the factors that influenced whether “MM or better status” or “MM or better status with an oral prednisolone (PSL) dose of 5 mg/day or less (5-mg MM)” was met in patients with acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis (MG).
Methods
We performed a retrospective study in 93 patients with AChR antibody-positive generalized MG who were followed for 3 years after the start of immunotherapy. We reviewed clinical data, such as MG-related symptoms, the MG activities of daily living profile (MGADL) score, immunotherapy including the dose of PSL, and achievement of the status of MM or better at baseline and 3, 6, 12, 24, and 36 months after treatment.
Results
An MM or better status was achieved in 60% of the patients 3 months and in 90% of the patients 2 years after initiating immunotherapy. At 2 years, 60% of the patients had achieved the treatment goal, which was an “5-mg MM”. More frequent plasmapheresis and higher dose of PSL within 3 months after immunotherapy initiation were associated with difficulty in achieving the 5-mg MM status at 2 years.
Conclusion
Approximately 60% of the MG patients achieved the treatment goal within 2 years after treatment. PSL dose and the cumulative number of plasmapheresis procedures at 3 months after immunotherapy initiation may help identify treatment-resistant patients with MG.
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Data availability
Any data not published within the article will be shared anonymized upon request from any qualified investigator.
Abbreviations
- MG:
-
Myasthenia gravis
- AChR:
-
Acetylcholine receptor
- MGADL score:
-
MG activities of daily living score
- PSL:
-
Prednisolone
- IVIg:
-
Intravenous immunoglobulin
- PP:
-
Plasmapheresis
- CNIs:
-
Calcineurin inhibitors
- IVMP:
-
High-dose intravenous methyl-prednisolone
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Acknowledgements
The author would like to thank Enago (www.enago.jp) for the English language review.
Funding
This work was supported in part by the Health and Labour Sciences Research Grant on Intractable Diseases (Neuroimmunological Diseases) from the Ministry of Health, Labour and Welfare of Japan (20FC1030).
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YO, AU, and SK made substantial contribution to the conception and design of study. YO, AU, MY, YK, YOn, FO, TK, KH and NK collected the data. YO and TK conducted the statistical analysis. YO drafted the manuscript; All authors were involved in drafting the article or critically revising its important intellectual content. All authors have read and approved the final version of the manuscript..
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The authors declare no financial or other conflicts of interest. All authors report no disclosures.
Ethical statement
The ethics committee of our institute approved this study (IRB No. 2266). The investigation conforms to the principles outlined in the Declaration of Helsinki in relation to the ethical principles for medical research involving human subjects.
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Ozawa, Y., Uzawa, A., Yasuda, M. et al. Long-term outcomes and prognostic factors in generalized myasthenia gravis. J Neurol 268, 3781–3788 (2021). https://doi.org/10.1007/s00415-021-10520-x
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DOI: https://doi.org/10.1007/s00415-021-10520-x