Abstract
Introduction
Intravenous immunoglobulin (IVIg) has been proven beneficial in myasthenic crisis, but their role as maintenance therapy is unclear. The aim of this study was to determine if maintenance therapy with low-dose IVIg improves clinical outcome and may be used as a steroid-sparing agent in myasthenia gravis (MG).
Methods
We retrospectively reviewed charts of all MG patients treated with IVIg from January 2006 to December 2019. Long-term treatment response to IVIg was assessed by improvement in the Myasthenia Gravis Foundation of America (MGFA) clinical classification scale as primary end point, as well as the ability to reduce the time-weighted average required dose of prednisone as secondary end-point, in a follow-up period of 36 months.
Results
109 patients were treated with IVIg. The mean follow-up time was 34.03 ± 5.5 months. Sixty-seven patients (61.4%) responded to therapy with at least one-point improvement of the MGFA scale. There was no statistical difference in demographic and clinical characteristics between IVIg responders and non-responders. The mean prednisone dose decreased significantly from 33.1 ± 14.5 mg at baseline to 7.2 ± 7.8 mg after 36 months of IVIg treatment (P < 0.0001), with the greatest effect after 6 months (33.1 ± 14.5 mg Vs. 17.9 ± 11.7 mg; P < 0.0001). In the follow-up period of 36 months, most patients (92.5%) remained clinically and pharmacologically stable under chronic IVIg treatment.
Conclusion
This retrospective study demonstrates that chronic low-dose IVIg treatment in patients with MG improves clinical outcomes and has a prolonged and significant steroid-sparing effect over a period of 3 years.
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Availability of data and material
The data presented in this report will be made available to bona fide investigators upon request to the corresponding author.
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Adi Wilf-Yarkoni and Mark A. Hellmann contributed to the study conception and design. Material preparation, data collection and analysis were performed by Adi Wilf-Yarkoni and Mark A. Hellmann. The first draft of the manuscript was written by Adi Wilf-Yarkoni and Mark A. Hellmann. All authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
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All data gathered after approval by the Ethics Committee of Rabin Medical Center and therefore been performed in accordance with the ethical standards laid down in the 1964 declaration of Helsinki.
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Wilf-Yarkoni, A., Lotan, I., Steiner, I. et al. Chronic low-dose intravenous immunoglobulins as steroid-sparing therapy in myasthenia gravis. J Neurol 268, 3871–3877 (2021). https://doi.org/10.1007/s00415-021-10544-3
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DOI: https://doi.org/10.1007/s00415-021-10544-3