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Chronic low-dose intravenous immunoglobulins as steroid-sparing therapy in myasthenia gravis

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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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Funding

This work did not receive any funding.

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Authors and Affiliations

  1. Department of Neurology, Rabin Medical Center, Beilinson Campus, 49100, Petach Tikva, Israel

    Adi Wilf-Yarkoni, Itay Lotan, Israel Steiner & Mark A. Hellmann

  2. Neuroimmunology Unit, Rabin Medical Center, Beilinson Hospital, Petach Tikva, Israel

    Adi Wilf-Yarkoni, Itay Lotan & Mark A. Hellmann

  3. Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel

    Adi Wilf-Yarkoni, Itay Lotan, Israel Steiner & Mark A. Hellmann

Authors
  1. Adi Wilf-Yarkoni
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  2. Itay Lotan
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  3. Israel Steiner
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  4. Mark A. Hellmann
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Contributions

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.

Corresponding author

Correspondence to Adi Wilf-Yarkoni.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

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.

Consent to participate

The Institutional Review Board for human experiments waived informed consent.

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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

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