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Expert Review of Clinical Immunology Volume 12, 2016 - Issue 2

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Overcoming challenges in the diagnosis and treatment of myasthenia gravis

Amelia EvoliInstitute of Neurology, Catholic University, Roma, ItalyCorrespondence[email protected]

Raffaele IorioInstitute of Neurology, Catholic University, Roma, Italy;Don Gnocchi ONLUS Foundation, Milan, Italy

Emanuela BartoccioniInstitute of General Pathology, Catholic University, Roma, Italy

Pages 157-168 | Received 31 Aug 2015, Accepted 16 Oct 2015, Published online: 16 Dec 2015

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https://doi.org/10.1586/1744666X.2016.1110487
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Overcoming challenges in the diagnosis and treatment of myasthenia gravis

References

** The authors demonstrate that human IgG4 muscle-specific tyrosine kinase receptor (MuSK) antibodies bind to the first Ig-like domain in MuSK, thus disrupting MuSK–low-density lipoprotein receptor-related protein 4 interaction and inhibiting agrin-stimulated MuSK phosphorylation.

* A comprehensive review of antibodies to muscle antigens in myasthenia gravis. The authors thoroughly discuss the relevance of different antibodies in diagnosis and as measure of disease severity.

* This study reports the clinical correlates of clustered-acetylcholine receptor antibodies, showing a high rate of positive results in patients with childhood or ocular myasthenia gravis.

** In this passive transfer study, pyridostigmine exacerbated the structural and functional alterations induced by muscle-specific tyrosine kinase receptor antibodies at motor endplate. In contrast, 3-4 diaminopyridine enhanced neuromuscular transmission.

* This study provides Class I evidence that intravenous immunoglobulin and plasma-exchange have comparable efficacy in patients with moderate to severe myasthenia gravis.

* This review describes how long-lived plasma cells survive in a protected microenvironment and discusses the strategies for their specific depletion.

* This study demonstrates responsiveness in myasthenia gravis patients was associated with a rapid repopulation of B regulatory cells.

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Overcoming challenges in the diagnosis and treatment of myasthenia gravis

References

** The authors demonstrate that human IgG4 muscle-specific tyrosine kinase receptor (MuSK) antibodies bind to the first Ig-like domain in MuSK, thus disrupting MuSK–low-density lipoprotein receptor-related protein 4 interaction and inhibiting agrin-stimulated MuSK phosphorylation.

* A comprehensive review of antibodies to muscle antigens in myasthenia gravis. The authors thoroughly discuss the relevance of different antibodies in diagnosis and as measure of disease severity.

* This study reports the clinical correlates of clustered-acetylcholine receptor antibodies, showing a high rate of positive results in patients with childhood or ocular myasthenia gravis.

** In this passive transfer study, pyridostigmine exacerbated the structural and functional alterations induced by muscle-specific tyrosine kinase receptor antibodies at motor endplate. In contrast, 3-4 diaminopyridine enhanced neuromuscular transmission.

* This study provides Class I evidence that intravenous immunoglobulin and plasma-exchange have comparable efficacy in patients with moderate to severe myasthenia gravis.

* This review describes how long-lived plasma cells survive in a protected microenvironment and discusses the strategies for their specific depletion.

* This study demonstrates responsiveness in myasthenia gravis patients was associated with a rapid repopulation of B regulatory cells.

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