Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture

Koneczny, Inga; Herbst, Ruth

doi:10.3390/cells8070671

Cited by 120 publications

(103 citation statements)

References 354 publications

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“…AChRAbs are present in the 40-90% of patients with MG and allow, together with other antibodies (against MuSK, Lrp4, agrin, etc. ), the subclassification of different 9 types of MG, particularly the first 4 types (early onset MG, late onset MG, thymoma-associated MG, and ocular MG) [39]. These autoantibodies induce pathogenicity by three main mechanisms: activation of the classical complement cascade, endocytosis with loss of AChR density, and direct inhibition of AChR binding of ACh or blocking the ACh channel [39].…”

Section: Myasthenia Gravis and Acetylcholine Receptormentioning

confidence: 99%

“…), the subclassification of different 9 types of MG, particularly the first 4 types (early onset MG, late onset MG, thymoma-associated MG, and ocular MG) [39]. These autoantibodies induce pathogenicity by three main mechanisms: activation of the classical complement cascade, endocytosis with loss of AChR density, and direct inhibition of AChR binding of ACh or blocking the ACh channel [39]. There are several reliable diagnostic assays to detect autoantibodies against AChR, including RIA and ELISA [40].…”

Section: Myasthenia Gravis and Acetylcholine Receptormentioning

confidence: 99%

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Receptor autoimmunity: diagnostic and therapeutic implications

Tozzoli¹

2020

Autoimmun Highlights

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Receptor autoimmunity is one of the ways in which autoimmune diseases appear in humans. Graves' disease, myasthenia gravis, idiopathic membranous nephropathy, and autoimmune acute encephalitis are the major autoimmune diseases belonging to this particular group. Receptor autoimmune disease are dependent on the presence of autoantibodies directed against cell-surface antigens, namely TSH receptor in thyrocytes, acetylcholine receptor in neuromuscular junction, phospholipase 2 receptor in podocytes, and NMDA receptor in cortical neurons. In this article we outline the distinctive features of receptor autoimmunity and the specific relationship between the autoimmunology laboratory and the presence/concentration of autoantibodies. Some immunological features distinguish receptor autoimmunity. Anti-receptor autoantibody pathologies are considered T cell-dependent, B-cell-mediated autoimmune disorders: the knowledge about the presence of circulating and/or localized autoantibodies to target organs and identification of autoantigens involved in the autoimmune reaction is of paramount importance. Due to the close correlation between the concentration of anti-receptor autoantibodies, the autoimmune target of some cell-surface receptors and the intensity of symptoms, the measurement of these immunoglobulins has become central to diagnose autoimmune diseases in all affected patients, not just in clinically dubious cases. The measurement of autoantibodies is also relevant for differential diagnosis of autoimmune and non-autoimmune forms with similar symptoms. From the methodological point of view, quantitative immunoassay methods of measurement should be preferred over semi-quantitative ones, for the capacity of the first class of methods to define precisely the reference ranges and decision levels overcoming the measurement uncertainty of semi-quantitative methods.

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Section: Myasthenia Gravis and Acetylcholine Receptormentioning

confidence: 99%

Section: Myasthenia Gravis and Acetylcholine Receptormentioning

confidence: 99%

Receptor autoimmunity: diagnostic and therapeutic implications

Tozzoli¹

2020

Autoimmun Highlights

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“…MG is rare chronic disorder, and is considered as a classic example of an anti-mediated autoimmune disease, with the characterizing symptom of localized or general muscle weakness 12 . In most cases, MG still requires chronic immunotherapy with immunosuppressive drugs to maintain disease control 13 .…”

Section: Discussionmentioning

confidence: 99%

The impact of COVID-19 epidemic on adult Myasthenia Gravis patients

Peng

Zeng

et al. 2020

Preprint

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Objects: To investigate the impact of COVID-19 epidemic on adult myasthenia gravis (MG) patients, in order to provide better medical advice for special patient population during the quarantine period.Methods: Adult MG patients were randomly recruited to participate. The survey consisted of self-designed questionnaires and the revised 15-item Myasthenia Gravis Quality of Life Questionnaire (MGQOL15r). Participation was strictly voluntary and remained anonymous.Results: A total of 214 adult MG patients (84 males and 130 females) gave valid replies. There were 49.53% patients reported they had the fear of COVID-19 outbreak at different levels. And female patients had a significant higher level of fear to COVID outbreak (p=0.009). It was found that the adult MG patients are more likely to be influenced by the COVID-19 epidemic compared to the general population. During the COVID-19 outbreak, 36.45% patients reported they got an improvement of MG disease severity. 57.01% patients reported they felt no change. 6.54% patients reported their condition got worse. The average of MGQOL15r score was 7.38±6.22. The higher level of the fear of COVID-19 outbreak, the higher score of MGQOL15r (p=0.029), the poorer quality of life. 96.73% patients hoped the specialist physicians could provide online consult. In addition, 64.95% patients would go to the tertiary hospital without hesitation if the Outpatients Department was opened even during the COVID-19 epidemic.Conclusions: The COVID-19 epidemic has significant impact on the quality of life and psychological status in the adult MG patients. Compared to the general population, the adult MG patients are more likely to be influenced by the COVID-19 epidemic. Hence, it is important for health care organizations to provide professional therapeutic advice and psychosocial support in time.*, Contributed to this work equally.

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“…Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disorder. Patients with MG who are seropositive for autoantibodies to the acetylcholine receptor (AChR), musclespecific tyrosine kinase (MuSK), or low-density lipoprotein receptor-related protein 4 (LRP4) present with voluntary muscle weakness due to dysfunctional neuromuscular junctions and impaired neuromuscular transmission (1,2). Traditional therapies for MG including thymectomy, intravenous immunoglobulin (IVIg), plasmapheresis, and corticosteroid therapy can induce remission, but do not cure the disease.…”

Section: Introductionmentioning

confidence: 99%

New Approaches to Targeting B Cells for Myasthenia Gravis Therapy

Huda

2020

Front. Immunol.

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Current therapies for myasthenia gravis (MG) are limited, and many investigations have recently focused on target-specific therapies. B cell-targeting monoclonal antibody (mAb) therapies for MG are increasingly attractive due to their specificity and efficacy. The targeted B cell biomarkers are mainly the cluster of differentiation (CD) proteins that mediate maturation, differentiation, or survival of pathogenic B cells. Additional B cell-directed therapies include non-specific peptide inhibitors that preferentially target specific B cell subsets. The primary goals of such therapies are to intercept autoantibodies and prevent the generation of an inflammatory response that contributes to the pathogenesis of MG. Treatment of patients with MG using B cell-directed mAbs, antibody fragments, or selective inhibitors have exhibited moderate to high efficacy in early studies, and some of these therapies appear to be highly promising for further drug development. Numerous other biologics targeting various B cell surface molecules have been approved for the treatment of other conditions or are either in clinical trials or preclinical development stages. These approaches remain to be tested in patients with MG or animal models of the disease. This review article provides an overview of B cell-targeted treatments for MG, including those already available and those still in preclinical and clinical development. We also discuss the potential benefits as well as the shortcomings of these approaches to development of new therapies for MG and future directions in the field.

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Myasthenia Gravis: Pathogenic Effects of Autoantibodies on Neuromuscular Architecture

Cited by 120 publications

References 354 publications

Receptor autoimmunity: diagnostic and therapeutic implications

Receptor autoimmunity: diagnostic and therapeutic implications

The impact of COVID-19 epidemic on adult Myasthenia Gravis patients

New Approaches to Targeting B Cells for Myasthenia Gravis Therapy

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