Abstract
Autoimmune myasthenia gravis (MG) is a neuromuscular junction disorder marked clinically by fatigable muscle weakness and serologically by the presence of autoantibodies against acetylcholine receptors (AChRs), muscle-specific kinase (MuSK), or lipoprotein-related protein 4 (LPR4). Over the past few decades, the mortality of patients with MG has seen a dramatic decline secondary to evolving interventions in critical care and medical management. In the past 2 to 3 years, there have been several changes in standard of care for the treatment of MG. These changes include confirmation of the benefit of thymectomy versus medical management alone in AChR patients and a new US Food and Drug Administration-approved medication for refractory MG. There are also several exciting new prospective drugs in the pipeline, which are in different stages of clinical trial testing.
Highlights
Autoimmune myasthenia gravis (MG) is a neuromuscular junction (NMJ) disorder marked clinically by fatigable muscle weakness and serologically by the presence of autoantibodies
In musclespecific kinase (MuSK) MG, which accounts for about 10% of the population of patients with MG, antibodies bind to the Ig-like region, blocking activation of the agrin–LRP4–MuSK complex and inhibiting neuromuscular transmission[8]
Results showed that patients randomly assigned to the thymectomy group did better clinically over the 3-year period, with a mean average improvement of almost 3 points (2.85) on the quantitative MG (QMG) score
Summary
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