Myasthenie.com

Muscle Nerve. 2021 May 19. doi: 10.1002/mus.27326. Online ahead of print.

ABSTRACT

INTRODUCTION: Eculizumab has been shown to be efficacious in acetylcholine receptor antibody positive (AChR+), Myasthenia Gravis Foundation of America (MGFA) Class II-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 score (MGC) and MG-Activity of Daily Living (MG-ADL) scores were assessed before and after eculizumab.

RESULTS: Two of 3 gMG patients, refractory to IVIG, plasmapheresis, prednisone and (in one) rituximab, showed a robust response to eculizumab with marked improvement in MGFA, MG-ADL and MGC. The third patient showed a partial response to eculizumab but remained on noninvasive ventilation (NIV) and gastrostomy-tube. Patients 1 and 2 achieved minimal manifestation (MM) status at week 4 and week 6 respectively and showed continued improvement in MG-ADL and MGC through week 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 afterwards in MG-ADL and MGC, possibly due to delay in his 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. This article is protected by copyright. All rights reserved.

PMID:34008175 | DOI:10.1002/mus.27326


Source: https://pubmed.ncbi.nlm.nih.gov/3400817 ... 5&v=2.14.4