Journal Article

Independent risk factors for myasthenic crisis and disease exacerbation in a retrospective cohort of myasthenia gravis patients

Mergenthaler, Philipp, Arat, Ercan, Vielhaber, Stefan, Stascheit, Frauke, Schroeter, Christina B., Meuth, Sven G., Pawlitzki, Marc, Totzeck, Andreas, Eckert, Carmen, Nelke, Christopher, Huntemann, Niklas, Öztürk, Menekse, Hagenacker, Tim, Schreiber, Stefanie, Gassa, Asmae, Schroeter, Michael, Meisel, Andreas, Foell, Dirk, Berger, Benjamin, Ruck, Tobias, Wiendl, Heinz, Stetefeld, Henning

2022

Overview

Background Myasthenic crisis (MC) and disease exacerbation in myasthenia gravis (MG) are associated with significant lethality and continue to impose a high disease burden on affected patients. Therefore, we sought to determine potential predictors for MC and exacerbation as well as to identify factors affecting outcome. Methods We examined a retrospective, observational cohort study of patients diagnosed with MG between 2000 and 2021 with a mean follow-up of 62.6 months after diagnosis from eight tertiary hospitals in Germany. A multivariate Cox regression model with follow-up duration as the time variable was used to determine independent risk factors for MC and disease exacerbation. Results 815 patients diagnosed with MG according to national guidelines were included. Disease severity at diagnosis (quantitative MG score or Myasthenia Gravis Foundation of America class), the presence of thymoma and anti-muscle specific tyrosine kinase-antibodies were independent predictors of MC or disease exacerbation. Patients with minimal manifestation status 12 months after diagnosis had a lower risk of MC and disease exacerbation than those without. The timespan between diagnosis and the start of immunosuppressive therapy did not affect risk. Patients with a worse outcome of MC were older, had higher MGFA class before MC and at admission, and had lower vital capacity before and at admission. The number of comorbidities, requirement for intubation, prolonged mechanical ventilation, and MC triggered by infection were associated with worse outcome. No differences between outcomes were observed comparing treatments with IVIG (intravenous immunoglobulin) vs. plasma exchange vs. IVIG together with plasma exchange. Conclusions MC and disease exacerbations inflict a substantial burden of disease on MG patients. Disease severity at diagnosis and antibody status predicted the occurrence of MC and disease exacerbation. Intensified monitoring with emphasis on the prevention of infectious complications could be of value to prevent uncontrolled disease in MG patients. Graphical Abstract

• Publisher: BioMed Central,BioMed Central Ltd,Springer Nature B.V,BMC
• Subject: Antibodies / Biomedical and Life Sciences / Biomedicine / Development and progression / Diagnosis / Disease exacerbation / Disease Progression / Documentation / Humans / Immunoglobulins / Immunoglobulins, Intravenous - therapeutic use / Immunology / Immunosuppressive agents / Intensive care / Intravenous administration / Intubation / Lethality / Mechanical ventilation / Medical prognosis / Myasthenia / Myasthenia gravis / Myasthenia Gravis - complications / Myasthenia Gravis - epidemiology / Myasthenia Gravis - therapy / Myasthenic crisis / Neurobiology / Neurology / Neuromuscular junctions / Neurosciences / Patients / Predictors / Protein-tyrosine kinase / Retrospective Studies / Risk Factors / Statistical analysis / Thymoma / Variance analysis