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.
Huijbers, M. G., Vink, A. F. D., Niks, E. H., Westhuis, R. H., Van Zwet, E. W., De Meel, R. H., Rojas García, R., Díaz Manera, J., Kuks, J. B., Klooster, R., Straasheijm, K., Evoli Stampanoni-B, A., Illa, I., Van Der Maarel, S. M., Verschuuren, J. J., Longitudinal epitope mapping in MuSK myasthenia gravis: Implications for disease severity, <<JOURNAL OF NEUROIMMUNOLOGY>>, 2016; 291 (Febbraio): 82-88. [doi:10.1016/j.jneuroim.2015.12.016] [http://hdl.handle.net/10807/94711]
Longitudinal epitope mapping in MuSK myasthenia gravis: Implications for disease severity
Evoli Stampanoni-B, Amelia;
2016
Abstract
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.
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