Journal
JOURNAL OF NEUROIMMUNOLOGY
Volume 291, Issue -, Pages 82-88Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.jneuroim.2015.12.016
Keywords
MuSK; Myasthenia gravis; Neuromuscular junction; Epitope mapping; IgG4
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Funding
- Prinses Beatrix Spierfonds
- Association Francaise contre les Myopathies
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Muscle weakness in MuSK myasthenia gravis (MG) is caused predominantly by IgG4 antibodies which block MuSK signalling and destabilize neuromuscular junctions. We determined whether the binding pattern of MuSK IgG4 antibodies change throughout the disease course (epitope spreading), and affect disease severity or treatment responsiveness. We mapped the MuSK epitopes of 255 longitudinal serum samples of 53 unique MuSK MG patients from three independent cohorts with ELISA. Antibodies against the MuSK Iglike-1 domain determine disease severity. Epitope spreading outside this domain did not contribute to disease severity nor to pyridostigmine responsiveness. This provides a rationale for epitope specific treatment strategies. (C) 2016 Elsevier B.V. All rights reserved.
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