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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8250645PMC
http://dx.doi.org/10.1111/ene.14827DOI Listing

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Background: Increasing data are available on the use and efficacy of rituximab (RTX) in patients with anti-muscle-specific tyrosine kinase (MuSK)-positive myasthenia gravis (MG), especially those steroid-dependent or unresponsive to traditional immunotherapies.

Aims: We aimed to evaluate the clinical characteristics and treatment responses of adult patients with generalized anti-MuSK-positive MG treated with RTX.

Methods: We retrospectively recruited 16 patients who were on RTX, between January 2010 and September 2023.

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Introduction And Importance: The primary clinical symptom in people with myasthenia gravis (MG) is muscle weakness that gets worse with activity and gets better with rest; often, the first symptoms are ocular ones, such as ptosis and double vision. On the other hand, individuals with anti-muscle-specific tyrosine kinase may present with unusual symptoms. Nonetheless, it is hypothesized that muscle-specific tyrosine kinase antibodies may be present when no antibodies are present, along with bulbar and respiratory symptoms.

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Muscle-specific kinase (MuSK) Myasthenia Gravis (MG) represents a prototypical antibody-mediated disease characterized by predominantly focal muscle weakness (neck, facial, and bulbar muscles) and fatigability. The pathogenic antibodies mostly belong to the immunoglobulin subclass (Ig)G4, a feature which attributes them their specific properties and pathogenic profile. On the other hand, acetylcholine receptor (AChR) MG, the most prevalent form of MG, is characterized by immunoglobulin (Ig)G1 and IgG3 antibodies to the AChR.

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Myasthenia gravis (MG) is an autoimmune disease that is characterised by the formation of antibodies against acetylcholine receptors in the postsynaptic membrane of the neuromuscular junction. The course of the disease cannot be predicted during pregnancy. A subtype of MG with positive muscle-specific receptor tyrosine kinase (anti-MuSK) antibodies exhibits more localised clinical characteristics and a poor response to treatment compared with the disease subtype that involves positivity for acetylcholine receptor antibodies.

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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.

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