C3, C5a and anti-acetylcholine receptor antibody as severity biomarkers in myasthenia gravis

Aguirre, Florencia; Manín, Analisa; Fernández, Victoria; Justo, Mariano E; Leoni, Juliana; Paz, Mariela L.; Villa, Andrés

doi:10.1177/1756286420935697

Cited by 20 publications

(27 citation statements)

References 34 publications

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“…Other studies have also shown a correlation of MuSK-AB-titer and MG severity in general [ 20 ]. Aguirre et al [ 21 ] demonstrated similar results; they found a relation between AchR-AB titers and disease severity in the first 5 years of MG as well as they detected complement factor C5a significantly elevated in severe disease courses as a non-specific marker. Up to our knowledge, only a few studies, especially case reports , exist that repetitively measured antibody titers during disease course.…”

Section: Discussionmentioning

confidence: 67%

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

et al. 2021

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Myasthenic crisis (MC) is a life-threatening condition for patients with myasthenia gravis (MG). Muscle-specific kinase-antibodies (MuSK-ABs) are detected in ~ 6% of MG, but data on outcome of MuSK-MCs are still lacking. We made a subgroup analysis of patients who presented with MC with either acetylcholine-receptor-antibody positive MG (AchR-MG) or MuSK-MG between 2006 and 2015 in a retrospective German multicenter study. We identified 19 MuSK-AB associated MCs in 15 patients and 161 MCs in 144 patients with AchR-ABs only. In contrast to patients with AchR-AB, MuSK-AB patients were more often female (p = 0.05, OR = 2.74) and classified as Myasthenia Gravis Foundation of America-class IV before crisis (p = 0.04, OR = 3.25). MuSK-AB patients suffer more often from multiple chronic disease (p = 0.016, OR = 4.87) and were treated more invasively in terms of plasma exchanging therapies (not significant). The number of days of mechanical ventilation (MV) (43.0 ± 53.1 vs. 17.4 ± 18; p < 0.0001), days on an intensive care unit (ICU) (45.3 ± 49.5 vs. 21.2 ± 19.7; p < 0.0001), and hospital-length of stay (LOS) (55.9 ± 47.6 vs. 28.8 ± 20.9 days; p < 0.0001) were significantly increased in MuSK-MC. Remarkable is that these changes were mainly due to patients with MusK-ABs only, whereas patients’ outcome with both antibodies was similar to AchR-MCs. Furthermore, our data showed a shortened duration of MV after treatment with plasma exchanging therapies compared to treatment with intravenous immunoglobulin in MuSK-MCs. We conclude that MuSK-AB-status is associated with a longer need of MV, ICU-LOS, and hospital-LOS in MC, and therefore recommend early initiation of a disease-specific therapy.

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Section: Discussionmentioning

confidence: 67%

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

et al. 2021

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“…After treatment, the indices returned to their original baseline levels. Interestingly, C5a levels were positively correlated with MG severity according to the activities of daily living (ADL) scale (32). Components of the complement system can be considered as markers for diagnosis and disease, as well as targets for therapy.…”

Section: The Complement System In Mgmentioning

confidence: 99%

Clinical Efficacy and Safety of Eculizumab for Treating Myasthenia Gravis

Xiao

Liang

et al. 2021

Front. Immunol.

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Myasthenia gravis (MG) is an autoimmune disease primarily mediated by acetylcholine receptor antibodies (AChR-Ab), cellular immune dependence, and complement system involvement. Since the AChR on the postsynaptic membrane is destroyed by an immune attack, sufficient endplate potential cannot be generated, resulting in the development of a synaptic transmission disorder at the neuromuscular junction and in muscle weakness. The role of the complement system in MG has been demonstrated in animal models and clinical tests, and it has been determined that complement inhibition in patients with MG can prevent disease induction and reverse its progression. Eculizumab is a humanized monoclonal antibody that inhibits the cleavage of complement protein C5 and prevents autoimmune damage; additionally, it has received subsequent approval by the Federal Drug Administration of the United States for MG treatment. However, various concerns regarding the use of eculizumab persist. In this review, we have discussed the treatment time, cost effectiveness, long-term efficacy, and tolerability of eculizumab for MG treatment. We have also summarized historical information and have presented perspectives on this new therapeutic modality.

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“…The pathogenesis of MG is extremely complex and its occurrence is the result of the interaction between the genetic susceptibility of the host, the influence of environmental infectious factors, and the body's own immune response. AChR antibody [10][11], other major pathogenic autoantibodies, and THymus CD4+ T lymphocytes [12] play an important role in MG pathogenesis. Although the mechanism of MG relapse may be related to the β 2 adrenergic receptor, this mechanism is still unclear and rarely studied [13].…”

Section: Monofactorial Analysismentioning

confidence: 99%

Risk factors analysis and establishment of a clinical predictive model for recurrence after first attack of myasthenia gravis: a single-center retrospective study

Liu

Zhang

2021

Preprint

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Background Myasthenia gravis (MG) is a rare and recurrent disease. The purpose of this study was to investigate the risk factors for relapse in MG patients after their first attack and establish a clinical predictive model. We conducted a retrospective study of 86 MG patients, followed and reviewed the clinical data of patients from the first onset to the first relapse, including age of onset, site of first symptom, MGFA at onset, thymoma, surgical resection of the thymoma, infection history, irregular drug use, combination of other autoimmune diseases, AChR antibody, and anti-Musk antibody, etc. The R software was used for statistical analysis. Univariate analysis and multivariate analysis were used to analyze risk factors. The clinical predictive model was established by Logistic regression analysis. Results Within 2 years after the first attack, 61.2% of MG patients relapsed. MGFA at onset, irregular drug use and infection history were independent risk factors for MG relapse within 2 years after the first attack ( p < 0.05). The clinical prediction model has good discrimination and calibration. Conclusion The relapse of MG is affected by a variety of factors. The clinical predictive model that was established in this study can help clinicians predict the probability of relapse in MG patients, identify early high-risk relapse patients, and serve for high-quality clinical management.

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C3, C5a and anti-acetylcholine receptor antibody as severity biomarkers in myasthenia gravis

Cited by 20 publications

References 34 publications

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

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

Clinical Efficacy and Safety of Eculizumab for Treating Myasthenia Gravis

Risk factors analysis and establishment of a clinical predictive model for recurrence after first attack of myasthenia gravis: a single-center retrospective study

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