UB researcher co-chairs panel that revised, expanded myasthenia gravis guidelines

A UB researcher recently co-led a panel of 16 international experts on myasthenia gravis (MG) to revise and expand recommendations for managing the disease. Their paper was published in the journal Neurology on Nov. 3.

Gil I. Wolfe, Irvin and Rosemary Smith Chair of the Department of Neurology in the Jacobs School of Medicine and Biomedical Sciences at UB and president of UBMD Neurology, served as co-chair of the panel. He also co-chaired the same panel in 2016 when the guidelines were originally developed.

The new guidance for clinicians is based on the latest evidence in the literature. This updated formal consensus guidance provides recommendations to clinicians caring for MG patients worldwide.

“Some wealthy countries have established their own guidelines, but most of the world cannot do that,” Wolfe says. “The international panel, using the UCLA/RAND Appropriateness Methodology to achieve a formal consensus, hopes to fill that void, providing a treatment/management framework for health care providers, industry, insurers and the patient community.”

MG is a rare autoimmune disease affecting neuromuscular function. As many as 60,000 Americans have been diagnosed with MG, and its incidence is increasing as a result of improved diagnostic techniques and an aging population. Symptoms of MG include droopy eyelids; blurred or double vision; difficulty speaking, swallowing and breathing; and muscle weakness.

One of the main revisions to the recommendations encourages thymectomy (surgical removal of the thymus gland) in the largest subpopulation of MG patients. This change is based on a clinical trial for which Wolfe was the principal investigator. Results of that trial were published in the New England Journal of Medicine in 2016 and in The Lancet Neurology in 2019.

A new recommendation was also developed for the use of eculizimab, a complement inhibitor that is the first FDA-approved immunotherapy for MG. 

The panel also revised recommendations for the use of rituximab and methotrexate, as well as for early immunosuppression in ocular MG and MG associated with immune checkpoint inhibitor treatment.

In addition, there are some warnings in the recommendations pertaining to worsening MG clinical status in regard to certain therapies that have been touted for use in COVID-19, Wolfe says.

A list of panel participants and their institutions is available in the paper.