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B cells in the pathophysiology of myasthenia gravis
Author(s) -
Yi John S.,
Guptill Jeffrey T.,
Stathopoulos Panos,
Nowak Richard J.,
O’Connor Kevin C.
Publication year - 2018
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25973
Subject(s) - myasthenia gravis , autoantibody , immunology , autoimmunity , acetylcholine receptor , immunopathology , neuromuscular junction , autoimmune disease , pathophysiology , biology , neuroscience , medicine , receptor , immune system , pathology , antibody , genetics
Myasthenia gravis (MG) is an archetypal autoimmune disease. The pathology is characterized by autoantibodies to the acetylcholine receptor (AChR) in most patients or to muscle‐specific tyrosine kinase (MuSK) in others and to a growing number of other postsynaptic proteins in smaller subsets. A decrease in the number of functional AChRs or functional interruption of the AChR within the muscle end plate of the neuromuscular junction is caused by pathogenic autoantibodies. Although the molecular immunology underpinning the pathology is well understood, much remains to be learned about the cellular immunology contributing to the production of autoantibodies. This Review documents research concerning the immunopathology of MG, bringing together evidence principally from human studies with an emphasis on the role of adaptive immunity and B cells in particular. Proposed mechanisms for autoimmunity, which take into account that different types of MG may incorporate divergent immunopathology, are offered. Muscle Nerve 57 : 172–184, 2018