MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation

König, Nicole; Stetefeld, Henning; Dohmen, Christian; Mergenthaler, Philipp; Köhler, Siegfried; Schönenberger, Silvia; Bösel, Julian; Lee, Dong Hoon; Gerner, Stefan T.; Huttner, Hagen B.; Schneider, Hauke; Reichmann, Heinz; Fuhrer, Hannah; Berger, Benjamin; Zinke, Jan; Alberty, Anke; Kleiter, Ingo; Schneider‐Gold, Christiane; Roth, Christian; Dunkel, Juliane; Steinbrecher, Andreas; Thieme, Andrea; Schlachetzki, Felix; Linker, Ralf A.; Angstwurm, Klemens; Meisel, Andreas; Neumann, Bernhard

doi:10.1007/s00415-021-10603-9

Cited by 22 publications

(21 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Here, we investigated clinical features of seronegative MC compared to AChR-MC requiring mechanical ventilation based on our multicenter cohort of MC [ 2 ]. In contrast to MuSK-MCs, which are as old as AChR-MCs [ 5 ], we found that in seronegative MC, patients were younger but that the time between diagnosis of MG and onset of MC was longer compared to AChR-MC. Interestingly, 8 of 17 seronegative MCs had thymic abnormalities.…”

Section: Discussioncontrasting

confidence: 78%

See 1 more Smart Citation

Seronegative myasthenic crisis: a multicenter analysis

et al. 2022

Self Cite

View full text Add to dashboard Cite

Myasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Seronegative patients represent around 10–15% of MG, but data on outcome of seronegative MCs are lacking. We performed a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody-positive MG (AChR-MG) or seronegative MG between 2006 and 2015 in a retrospective German multicenter study. We identified 15 seronegative MG patients with 17 MCs and 142 AChR-MG with 159 MCs. Seronegative MCs were younger (54.3 ± 14.5 vs 66.5 ± 16.3 years; p = 0.0037), had a higher rate of thymus hyperplasia (29.4% vs 3.1%; p = 0.0009), and were more likely to be female (58.8% vs 37.7%; p = 0.12) compared to AChR-MCs. Time between diagnosis of MG and MC was significantly longer in seronegative patients (8.2 ± 7.6 vs 3.1 ± 4.4 years; p < 0.0001). We found no differences in duration of mechanical ventilation (16.2 ± 15.8 vs 16.5 ± 15.9 days; p = 0.94) and length of stay at intensive care unit (17.6 ± 15.2 vs 17.8 ± 15.4 days; p = 0.96), or in-hospital mortality (11.8% vs. 10.1%; p = 0.69). We conclude that MC in seronegative MG affects younger patients after a longer period of disease, but that crisis treatment efficacy and outcome do not differ compared to AChR-MCs.

show abstract

Section: Discussioncontrasting

confidence: 78%

“…Although seronegative MCs were less frequently treated with IVIg, there was no difference in other MC treatments. Furthermore, we did not find any difference in baseline characteristics, in the rate of complications or outcome between the patient groups, which is in contrast to more severely affected MuSK MC patients [ 5 ].…”

Section: Discussionmentioning

confidence: 98%

Seronegative myasthenic crisis: a multicenter analysis

et al. 2022

Self Cite

View full text Add to dashboard Cite

show abstract

“…Corroborating previous studies [ 25 , 26 ], we also identified anti-MuSK-ab positivity as an independent risk factor for disease deterioration. Anti-MuSK-ab positive has also been associated with poor outcome of MC [ 27 ]. Interestingly, disease severity as assessed by clinical scoring was a robust predictor for patients at risk for MC or exacerbation, underlining the importance of standardized clinical evaluation of MG patients.…”

Section: Discussionmentioning

confidence: 99%

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

Nelke

Stascheit

Eckert

et al. 2022

J Neuroinflammation

Self Cite

View full text Add to dashboard Cite

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

show abstract

“…MuSK antibody-positive MG was thought to have more severe symptoms, higher frequency of bulbar muscle and axial muscle involvement, and higher risk of developing myasthenic crisis. 21 , 22 Unlike anti-AChR antibody, MuSK antibodies are mostly IgG4. This subtype of IgG is functionally monovalent, with poor complement activation and low FcγR binding.…”

Section: Discussionmentioning

confidence: 99%

Factors affecting minimal manifestation status induction in myasthenia gravis

Yang

Dong

et al. 2022

Ther Adv Neurol Disord

View full text Add to dashboard Cite

Background: Minimal manifestation status (MMS) is an important landmark in the treatment of myasthenia gravis (MG), and predictors of MMS induction have rarely been identified in previous studies. Objective: The objective of this study is to evaluate the clinical factors associated with MMS induction among patients with MG. Design: This two-step retrospective cohort study with a single center investigated the factors that may be associated with MMS induction and retested these predictors in a test cohort. Methods: A total of 388 diagnosed MG patients who visited Xiangya Hospital between 1 July 2015 and 1 July 2019 were involved. We performed detailed chart reviews and recorded all cases achieving MMS. Demographics and clinical characteristics were also collected and their relationships to achieving MMS were investigated. Results: MMS was achieved in 124 patients (50.2%), and the median time to achieve MMS was 26 months. Several factors were found to be associated with MMS induction in exploring cohort, including muscle-specific tyrosine-protein kinase receptor (MuSK) antibody positivity (adjusted hazard ratio, HR = 4.333, 95% confidence interval, CI: 1.862–10.082), isolated ocular involvement (adjusted HR = 1.95, 95% CI: 1.284–2.961), and low baseline quantitative myasthenia gravis score (QMG score; adjusted HR = 2.022, 95% CI: 1.086–3.764). These factors were then retested in the test cohort. Isolated ocular involvement or low baseline QMG scores were factors found to be beneficial for MMS induction were confirmed. Conclusion: Isolated ocular involvement and low baseline QMG score are predictors of MMS induction in MG patients.

show abstract

MuSK-antibodies are associated with worse outcome in myasthenic crisis requiring mechanical ventilation

Cited by 22 publications

References 21 publications

Seronegative myasthenic crisis: a multicenter analysis

Seronegative myasthenic crisis: a multicenter analysis

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

Factors affecting minimal manifestation status induction in myasthenia gravis

Contact Info

Product

Resources

About