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

Amelia Evoli, 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, Isabel Illa, Silvère M. Van Der Maarel, Jan J. Verschuuren

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

30 Citazioni (Scopus)

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.
Lingua originaleEnglish
pagine (da-a)82-88
Numero di pagine7
RivistaJournal of Neuroimmunology
Volume291
DOI
Stato di pubblicazionePubblicato - 2016

Keywords

  • Adult
  • Aged
  • Aged, 80 and over
  • Autoantibodies
  • Cholinesterase Inhibitors
  • Enzyme-Linked Immunosorbent Assay
  • Epitope Mapping
  • Epitope mapping
  • Female
  • Humans
  • IgG4
  • Immunology
  • Immunology and Allergy
  • Italy
  • Longitudinal Studies
  • Male
  • Middle Aged
  • MuSK
  • Myasthenia Gravis
  • Myasthenia gravis
  • Neurology
  • Neurology (clinical)
  • Neuromuscular junction
  • Receptor Protein-Tyrosine Kinases
  • Receptors, Cholinergic
  • Severity of Illness Index
  • Spain
  • Statistics as Topic
  • Young Adult

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