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. 2021 Aug;64(2):212-215.
doi: 10.1002/mus.27326. Epub 2021 Jun 9.

The use of eculizumab in ventilator-dependent myasthenia gravis patients

Uzma Usman  1 , Christina Chrisman  2 , Drew Houston  3 , Clara Chow Haws  4 , Alan Wang  2 , Suraj Muley  3
Affiliations

The use of eculizumab in ventilator-dependent myasthenia gravis patients

Uzma Usman et al. Muscle Nerve. 2021 Aug.

Abstract

Introduction/aims: Eculizumab has been shown to be efficacious in acetylcholine receptor antibody-positive (AChR+ ) Myasthenia Gravis Foundation of America (MGFA) class II, III, and IV generalized myasthenia gravis (gMG) patients. However, it has not been studied in MGFA class V gMG patients.

Methods: We report three AChR+ , refractory, MGFA class V gMG patients treated with eculizumab. MGFA class, MG-Composite (MGC) score and MG Activities of Daily Living (MG-ADL) score were assessed before and after eculizumab.

Results: Two of three gMG patients, refractory to intravenous immunoglobulin, plasmapheresis, prednisone, and (in one case) rituximab, showed a robust response to eculizumab with marked improvement in MGFA, MG-ADL, and MGC measures. The third patient showed a partial response to eculizumab but remained on noninvasive ventilation and gastrostomy intubation. Patients 1 and 2 achieved minimal manifestation status at week 4 and week 6, respectively, and showed continued improvement on MG-ADL and MGC scores through weeks 55 and 43, respectively, with eculizumab. The third patient showed a partial response at week 10, followed by a slight decline in his MG-ADL score, but noted a slow but an incomplete improvement afterward on MG-ADL and MGC scores, possibly due to delayed eculizumab infusion.

Discussion: Eculizumab may play a role in the treatment of patients with MGFA class V, refractory gMG. Larger studies are required to provide further evidence.

Keywords: MGC, MGFA; eculizumab; myasthenia gravis; ventilator-dependent.

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References

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