Abstract

Background: Ten to fifteen percent of patients with myasthenia gravis (MG) have treatment-refractory disease. In short series and case reports, rituximab has proven to be effective in refractory MG.Methods: A retrospective, longitudinal study was conducted. Recruitment was performed in an MG cohort from a single third-level healthcare center in Mexico. The selection included refractory MG patients that were treated with rituximab. Response after rituximab therapy was assessed with MG composite score (MGCS) and prednisone dose reduction at 6, 12, and 18 months after initiation. Wilcoxon signed-rank test was used to evaluate differences between related groups for non-continual variables. P<0.05 was considered statistically significant.Results: Ten patients (7%) fulfilled criteria for refractory MG, and eight of them were treated with rituximab. The mean age at MG diagnosis was 25.5 (±2) years, with a female predominance (75%). All our patients (100%) had positive acetylcholine receptor (AchR) antibodies. The median MG duration was six years (interquartile range [IQR] 4.2-6) before rituximab initiation. All patients were previously treated with azathioprine and 50% additionally with cyclophosphamide. The median prednisone doses before rituximab treatment and 18-month follow-up were 50 mg (IQR 30-50 mg) and 10 mg (IQR 0-20 mg), respectively (p=0.011). The median baseline MGCS and at 18-month follow-up were 19.5 (IQR 11-31) and 6 (IQR0-16), respectively (p = 0.012).Conclusion: Rituximab appears to be associated with clinical improvement and prednisone dose reduction in Latin-American patients diagnosed with anti-AchR MG. Our findings need to be interpreted in light of the limitations mentioned.

Highlights

  • Published 02/08/2021License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.Myasthenia gravis (MG) is an antibody-mediated autoimmune disease characterized by fatigable muscle weakness

  • This study presents the experience with rituximab therapy in patients with refractory myasthenia gravis (MG) in a third-level healthcare center in Mexico

  • Ten patients (7%) fulfilled criteria for refractory MG, and eight of them were treated with rituximab

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Summary

Introduction

Myasthenia gravis (MG) is an antibody-mediated autoimmune disease characterized by fatigable muscle weakness It is the most frequent neuromuscular junction disorder, with an overall prevalence rate of 15-179 per million individuals [1]. Plasma exchange (PE) and intravenous immunoglobulin (IVIG) are recommended for myasthenic crisis, as well as severe presentations with bulbar muscle involvement, before thymectomy and to improve MG weakness before starting pharmacological therapies that take longer before an effect is observed. Both PE and IVIG are highly effective therapies but are expensive [3,4]. In short series and case reports, rituximab has proven to be effective in refractory MG

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