Management challenges in muscle-specific tyrosine kinase myasthenia gravis.

Amelia Evoli, Paolo Emilio Alboini, Emanuela Bartoccioni, Ana Bisonni, Alessia Mastrorosa

Risultato della ricerca: Contributo in rivistaArticolo in rivistapeer review

20 Citazioni (Scopus)

Abstract

Myasthenia gravis with antibodies to muscle-specific tyrosine kinase (MuSK-MG) is generally considered a severe disease because of the associated weakness distribution with prevalent involvement of bulbar muscles and a rapidly progressive course and early respiratory crises. Its treatment can be unrewarding, owing to poor response to acetylcholinesterase inhibitors in most patients, disease relapses in spite of high-dose immunosuppression, and development of permanent bulbar weakness. High-dose prednisone plus plasma exchange is the recommended approach for treating rapidly progressive bulbar weakness. In the disease management, oral steroids proved effective, plasma exchange produced marked, albeit short-term, improvement, while conventional immunosuppressants were comparatively less effective. Rituximab is a promising treatment for refractory MuSK-MG; in uncontrolled studies, nearly all treated patients achieved significant improvement with substantial decrease of medication. It is yet to be clarified whether the early use of rituximab could prevent the permanent bulbar weakness, which constitutes a relevant disability in these patients.
Lingua originaleEnglish
pagine (da-a)86-91
Numero di pagine6
RivistaAnnals of the New York Academy of Sciences
Volume2012
DOI
Stato di pubblicazionePubblicato - 2012

Keywords

  • anti-MuSK antibodies
  • myasthenia gravis

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