[pubmed] Performance of Different Criteria for Refractory Myasthenia Gravis

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Eur J Neurol. 2020 Dec 11. doi: 10.1111/ene.14675. Online ahead of print.

ABSTRACT

BACKGROUND: Defining refractory myasthenia gravis is important, as this can drive clinical decision-making, for example, by escalating treatments in refractory individuals. There are several definitions of refractory myasthenia and their performance has not been compared. Having valid and reliable criteria can help select patients in whom more aggressive treatments may be needed.

METHODS: We applied 5 different refractory myasthenia criteria (Drachman, Mantegazza, Suh, the International Consensus Guideline (ICG), and the REGAIN study) to a cohort of 237 patients. We compared the proportion of refractory patients among different criteria and their scores on disease severity, fatigue and quality of life scales. We also assessed the agreement for each criterion between two trained assessors.

RESULTS: The Drachman, Mantegazza, and Suh criteria resulted in high proportions of refractory individuals (40.1%, 39.2% and 38.8%, respectively), compared to the ICG and REGAIN criteria (9.7% and 3.0%, respectively). Refractory patients by the ICG and REGAIN criteria had worse disease severity, quality of life (QoL) and fatigue scores, compared to patients classified as refractory by other criteria. All criteria had high agreement between raters (between 70% and 100%).

CONCLUSIONS: There is high variability in the proportion of refractory MG patients depending on the criteria used, with ICG and REGAIN criteria capturing patients with worse disease severity. This reflects conceptual differences as to what refractory means. Further multi-centred studies are needed to determine appropriate criteria for refractory MG.

PMID:33306873 | DOI:10.1111/ene.14675


Source: https://pubmed.ncbi.nlm.nih.gov/3330687 ... 2&v=2.13.0