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Treatment of Myasthenia Gravis

Focus on Pyridostigmine

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Abstract

Acquired myasthenia gravis (MG) is a chronic autoimmune disorder of the neuromuscular junction, characterized clinically by muscle weakness and abnormal fatigability on exertion. Current guidelines and recommendations for MG treatment are based largely on clinical experience, retrospective analyses and expert consensus. Available therapies include oral acetylcholinesterase (AChE) inhibitors for symptomatic treatment, and short- and long-term disease-modifying treatments. This review focuses on treatment of MG, mainly on the use of the AChE inhibitor pyridostigmine. Despite a lack of data from well controlled clinical trials to support their use, AChE inhibitors, of which pyridostigmine is the most commonly used, are recommended as first-line therapy for MG. Pyridostigmine has been used as a treatment for MG for over 50 years and is generally considered safe. It is suitable as a long-term treatment in patients with generalized non-progressive milder disease, and as an adjunctive therapy in patients with severe disease who are also receiving immunotherapy. Novel AChE inhibitors with oral antisense oligonucleotides have been developed and preliminary results appear to be promising. In general, however, AChE inhibitors provide only partial benefit and most patients eventually switch to long-term immunosuppressive therapies, most frequently corticosteroids and/or azathioprine. Although AChE inhibitors are known to be well tolerated and effective in relieving the symptoms of MG, further efforts are required to improve treatment options for the management of this disorder.

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Acknowledgements

The authors thank Tracy Harrison of inScience Communications, a Wolters Kluwer business, who provided medical writing support funded by Meda Pharmaceuticals Inc., Solna, Stockholm, Sweden. Dr Mantegazza has received a speaker honorarium from Meda Pharmaceuticals Inc. Dr Maggi has no conflicts of interest that are directly relevant to the contents of this review.

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  1. Neurological Institute ‘Carlo Besta’, Via Celoria, 11, 20133, Milan, Italy

    Lorenzo Maggi &  Renato Mantegazza (Head of Neurology IV Department)

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  1. Lorenzo Maggi
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  2. Renato Mantegazza
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Correspondence to Renato Mantegazza.

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Maggi, L., Mantegazza, R. Treatment of Myasthenia Gravis. Clin. Drug Investig. 31, 691–701 (2011). https://doi.org/10.2165/11593300-000000000-00000

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