Abstract
Myasthenia gravis affects the neuromuscular junction of the skeletal muscles. It results in muscle weakness involving skeletal muscles (diaphragm, extraocular muscles) and myasthenic crisis. Treatment options for myasthenia gravis management have expanded, including azathioprine, corticosteroids, plasma exchange, and tacrolimus. Unfortunately, a few cases of myasthenia gravis don't respond to conventional treatment modalities. Monoclonal antibodies, rituximab (RTX), are novel treatments that have garnered interest as of late due to their efficacy within the patient population presented with refractory form myasthenia gravis. This review aims to showcase how RTX is an effective treatment within different forms of myasthenia gravis. A limited review was performed using databases that include PubMed and Google Scholar. The following keywords were used: "myasthenia gravis," "rituximab," "monoclonal antibody," "anti-AChR antibody," and "refractory myasthenia." The review focused on case reports, human studies, or research surveys based on the inclusion criteria of human studies involving participants more than 18 years of age and published in English literature. Out of 69 articles, 14 were duplicates, and 29 were relevant and met the inclusion criteria. The findings from the study demonstrate that patients with refractory myasthenia gravis responded well to RTX treatment. Furthermore, RTX has been shown to decrease corticosteroid dependence, induce sustained remission, and have a favorable response to anti-MuSK antibody positive myasthenia gravis compared to anti-AChR antibody positive myasthenia gravis. This literature review suggests that patients with refractory myasthenia gravis can benefit from rituximab; however, it has a variable response in different forms of myasthenia gravis.
Highlights
BackgroundMyasthenia gravis (MG) is an autoimmune disorder characterized by muscle weakness that worsens with exertion and improves with rest
This paper aims to determine the challenges and opportunities in the diagnosis of myasthenia gravis, to showcase the role of RTX in refractory generalized MG, to elucidate the differential response of RTX in muscle-specific tyrosine kinase (MuSK) antibody-positive MG as compared to acetylcholine receptor (AChR) antibody-positive MG, and to determine the role of RTX in inducing sustained remission in patients with refractory myasthenia gravis
Remission was achieved in 71.4% of the MuSK antibodypositive MG vs. 35.9% of the AChR antibody-positive patients (p = 0.022)
Summary
Myasthenia gravis (MG) is an autoimmune disorder characterized by muscle weakness that worsens with exertion and improves with rest. Extrinsic ocular muscle (EOM) weakness is the initial symptom in twothirds of patients, which progresses to involve bulbar muscles and limb muscles and later results in generalized MG. It is a rare disease with a prevalence of 20 per 100,000 individuals in the United States population [1]. Around 10-20% of patients with myasthenia gravis are found to be seronegative for AChR antibodies, and antibodies to the muscle-specific tyrosine kinase (MuSK) are found in 0-70% of MG patients [4]
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