Longitudinal epitope mapping in MuSK myasthenia gravis: Implications for disease severity

Maartje G. Huijbers*, Anna Fleur D. Vink, Erik H. Niks, Ruben H. Westhuis, Erik W. van Zwet, Robert H. de Meel, Ricardo Rojas-García, Jordi Díaz-Manera, Jan B. Kuks, Rinse Klooster, Kirsten Straasheijm, Amelia Evoli, Isabel Illa, Silvère M. van der Maarel, Jan J. Verschuuren

*Corresponding author for this work

Research output: Contribution to journalArticleResearchpeer-review

38 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)82-88
Number of pages7
JournalJournal of Neuroimmunology
Publication statusPublished - 15 Feb 2016


  • Epitope mapping
  • IgG4
  • MuSK
  • Myasthenia gravis
  • Neuromuscular junction


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