Epidemiology and 10-year clinical care of juvenile myasthenia gravis in England: a retrospective cohort study.

BACKGROUND

Published evidence is limited on the clinical burden of juvenile myasthenia gravis (JMG). We aimed to assess epidemiology and the clinical characteristics of JMG in England.

METHODS

We performed a retrospective analysis of patients with newly diagnosed JMG identified in England via primary care and hospital data between 2010 and 2019.

RESULTS

32 children (aged 2-17 years) with newly diagnosed JMG were included. Prevalence of JMG ranged from 2.2 (95% CI 1.5 to 3.1) in 2012 to 2.5 (95% CI 1.8 to 3.4) per 100 000 in 2018. The annual incidence ranged from 0.8 (95% CI 0.1 to 5.7) in 2015 to 3.8 (95% CI 1.6 to 9.0) per million per year in 2017. Incidence fluctuated in females from 1.6 (95% CI 0.2 to 11.3) in 2016 to 6 (95% CI 2.3 to 16.1) per million per year in 2018. Overall, 20 patients received first acetylcholinesterase inhibitors or corticosteroids with no prior therapy during the study period. During the follow-up period (median, 3.3 years), 17 patients (53.1%) with JMG experienced a hospitalisation. No deaths were observed.

CONCLUSIONS

This study confirms the rarity of JMG in England, with steady incidence and prevalence rates over a decade. Further research is required to assess unmet needs in JMG therapy and the importance of effective treatments for this condition.