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Hong et al. Neuroimmunol Neuroinflammation 2016;3:196-7 Neuroimmunology and
DOI: 10.20517/2347-8659.2016.33
Neuroinflammation

www.nnjournal.net
Commentary Open Access

Auto-reactive B cells in MuSK myasthenia

gravis

Yoon-Ho Hong , Jung-Joon Sung 2
1
1 Department of Neurology, Seoul National University College of Medicine, Seoul Boramae Medical Center, Seoul 07061, South Korea.
2 Department of Neurology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul 03080, South Korea.
Correspondence to: Prof. Jung-Joon Sung, Department of Neurology, Seoul National University College of Medicine, Seoul National University
Hospital, Seoul 03080, South Korea. E-mail: jjsant@snu.ac.kr

How to cite this article: Hong YH, Sung JJ. Auto-reactive B cells in MuSK myasthenia gravis. Neuroimmunol Neuroinflammation 2016;3:196-7.
Article history: Received: 22-05-2016 Accepted: 04-07-2016 Published: 31-08-2016

On [Guptill JT, Yi JS, Sanders DB, Guidon AC, Juel muscle, frequent myathenic crisis, poor outcome
VC, Massey JM, Howard JF Jr, Scuderi F, Bartoccioni with conventional immunosuppressants, intolerance
E, Evoli A, Weinhold KJ. Characterization of B cells to acetylcholinesterase inhibitors, and fewer thymic
in muscle-specific kinase antibody myasthenia gravis. pathologic changes. The disease is also known for
[6]
Neurol Neuroimmunol Neuroinflamm 2015;2:e77] its unique immunological features. The pathogenic
autoantibodies are mainly immunoglobulin G4
Acquired myasthenia gravis (MG) is a protoypical (IgG4), which unlike the IgG subtypes in AChR MG
[7]
autoimmune disease caused by a dysfunction of (IgG1 and IgG3) does not activate the complements
neuromuscular transmission at the postsynaptic part. and effector cells. Little is known about the precise
Patients experience fluctuating muscle weakness cellular components and molecular mechanisms in
that increases with exertion. It is typically classified MuSK MG.
into clinical subtypes depending on distribution of
[8]
involved muscles, onset age, thymic pathology, Guptill et al. have recently reported the characteristics
and auto-antibodies. While the most common auto- of B lymphocytes in MuSK MG patients. They
antibodies are targeted towards the skeletal muscle performed polychromatic flow cytometry and enzyme-
acetylcholine receptor (AChR), the list of target linked immuno sorbent assays in peripheral blood
molecules of pathogenic auto-antibodies has been samples from MuSK MG patients, and compared
expanding to include the muscle specific tyrosine immunological features of the patients to those
kinase (MuSK), low-density lipoprotein receptor- of healthy controls. They found no differences in
related protein 4 and agrin. [1-5] the frequencies of total B cells and B cell subsets
(naive, memory, class-switched, plasmablasts and
MuSK MG in particular has been of great interest. transitional cells) between the healthy controls and
It is clinically characterized by bulbar predominant MuSK MG patients who had not been treated with
manifestation, marked atrophy of the involved facial rituximab (anti-CD20 monoclonal antibody). There

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