Premium
Management challenges in muscle‐specific tyrosine kinase myasthenia gravis
Author(s) -
Evoli Amelia,
Alboini Paolo E.,
Bisonni Ana,
Mastrorosa Alessia,
Bartocccioni Emanuela
Publication year - 2012
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.2012.06781.x
Subject(s) - myasthenia gravis , weakness , rituximab , medicine , prednisone , muscle weakness , immunosuppression , refractory (planetary science) , tyrosine kinase , gastroenterology , surgery , lymphoma , biology , receptor , astrobiology
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.