Management of Myasthenia Gravis Around the Globe: Consensus Guidelines vs. Realities of Practice
Julia Greenberg1, Aravind Ganesh2, Kiril Kiprovski3, Sujata Thawani1
1Neurology, NYU Langone Hospital, 2Neurology, Department of Clinical Neurosciences, University of Calgary, 3Neurology, NYU-Langone Orthopedic Hospital
Objective:
To explore practices surrounding MG management across the globe with regard to alignment with the 2016 and 2020 international consensus guidelines.
Background:
There is great heterogeneity in the underlying pathophysiology of myasthenia gravis (MG), its clinical presentation, and response to treatment. International consensus guidelines were developed in 2016 and 2020 in an effort to standardize the approach to management of MG, however there remains uncertainty as to whether these efforts have been successful.
Design/Methods:
We administered an online, cross-sectional, case-based survey regarding MG management via Neurology’s: Practice Current section between 2020-2021 to neurologists and advanced practitioners. Analysis was purely descriptive. Responses were compared with recommendations outlined in the MG 2016 and 2020 consensus guidelines.
Results:
318 practitioners across 6 continents responded to the survey. Areas of discordance among respondents included the role of thymectomy, management of treatment-related side effects, management of acetylcholine receptor (AChR) antibody-negative MG, and management in preconception and pregnancy. Despite guidelines, only 54% of respondents recommended thymectomy in someone with AChR-MG, only 25% recommended vaginal delivery in someone with well controlled MG, and only 27% recommended early initiation of rituximab in someone with muscle-specific tyrosine kinase (MuSK) antibody-positive MG. Some of the areas of discordance observed were linked to regional differences in availability of therapies. For instance, 25% of respondents who did not recommended thymectomy indicated that thymectomy was not available to them in their current practice environment.
Conclusions:
Our preliminary survey data suggests that despite efforts to create a set of international consensus guidelines, uncertainty persists in the neurology community surrounding MG management. We identify areas of agreement and disagreement that can help clinicians refine their own practice patterns and define the need for modified guidelines that highlight the side effect profiles, cost, and availability of therapies worldwide.