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The use of eculizumab in ventilator‐dependent myasthenia gravis patients

Muscle & Nerve Jun 14, 2021

Usman U, Chrisman C, Houston D, et al. - Researchers describe the outcomes of three acetylcholine receptor antibody–positive, refractory, Myasthenia Gravis Foundation of America (MGFA) class V generalized myasthenia gravis (gMG) patients treated with eculizumab. A robust response to eculizumab along with marked improvement in MGFA, MG Activities of Daily Living (MG-ADL), and MG-Composite (MGC) measures was seen in two of three gMG patients refractory to intravenous immunoglobulin, plasmapheresis, prednisone, and (in one case) rituximab. A partial response to eculizumab was evident in the third patient but this patient remained on noninvasive ventilation and gastrostomy intubation. Minimal manifestation status was achieved by patients 1 and 2 at week 4 and week 6, respectively, and continued improvement on MG-ADL and MGC scores was exhibited by them through weeks 55 and 43, respectively, with eculizumab. At week 10, a partial response was seen in the third patient, followed by a slight decrease in his MG-ADL score, but he experienced a gradual but an incomplete improvement afterward on MG-ADL and MGC scores, likely because of delayed eculizumab infusion. Findings indicate a possible role of eculizumab in the treatment of patients with MGFA class V, refractory gMG.

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