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A panel of international myasthenia gravis (MG) experts provided new evidence-based recommendations for the management of MG, including updated guidance for thymectomy and medical treatment. The international consensus was published in Neurology.
A Task Force appointed by the Myasthenia Gravis Foundation of America included an international panel of MG experts that reviewed the previous 2016 consensus-based guidance for the management of MG. They then developed new and updated recommendations based on the latest evidence in the literature.
The consensus guidance includes a list of drugs to avoid or to use with caution in patients with MG due to potential worsening of MG. This list included aminoglycoside, fluoroquinolone, and macrolide antibiotics, beta-blockers, botulinum toxin, chloroquine and hydroxychloroquine, deferoxamine, and procainamide.
For patients with generalized MG who are between 18 to 50 years old and have antibodies to acetylcholine receptors (AChR), thymectomy should be considered early in the disease to improve clinical outcome, and to reduce immunotherapy requirements and hospitalization for exacerbations. The surgical intervention should be strongly considered when immunotherapy is ineffective or intolerable. The surgery should be performed when the patient is stable.
Immunosuppressant agents are the recommended treatment for ocular MG in patients with ophthalmoparesis or ptosis and functionally limiting or troublesome symptoms. Low dose corticosteroids may be effective, but steroid-sparing immunosuppressant agents may be required when corticosteroids alone are ineffective, contraindicated, or not tolerated.
Similar to the 2016 consensus guidance, rituximab should be considered early in the disease for patients with MG and muscle-specific kinase antibodies with an unsatisfactory response to initial immunotherapy, and can be considered when other immunosuppressive agents are ineffective or intolerable.
In patients with generalized MG, oral methotrexate may be used when other steroid-sparing agents that are better supported by randomized controlled trial data are ineffective or intolerable.
Eculizumab should be considered for patients with severe, refractory, generalized MG with AChR antibodies.
While a diagnosis of MG is not an absolute contraindication to the use of immune checkpoint inhibitors, the risk for MG and other immune-mediated neurologic complications should be discussed when this treatment is considered and close monitoring is required. Early aggressive treatment with high-dose steroids in combination with plasma exchange or intravenous immunoglobulin may be required in patients who develop overt MG while on immune checkpoint inhibitors.
The experts concluded that this consensus “continues to be a living document, which will require periodic review and updates to reflect new information relevant to the management of MG.”
Reference
Narayanaswami P, Sanders DB, Wolfe G, et al. International consensus guidance for management of myasthenia gravis: 2020 update. Neurology. 2021;96(3):114-122. doi:10.1212/WNL.0000000000011124
