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Anti‐musk antibody myasthenia gravis: Clinical findings and response to treatment in two large cohorts
Author(s) -
Guptill Jeffrey T.,
Sanders Donald B.,
Evoli Amelia
Publication year - 2011
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.22006
Subject(s) - myasthenia gravis , medicine , autoantibody , antibody , acetylcholine receptor , muscle weakness , weakness , gastroenterology , disease , immunology , receptor , surgery
Myasthenia gravis (MG) patients with autoantibodies to muscle‐specific tyrosine kinase (MuSK) represent a distinct subset of those with this disease. Treatment and outcomes data in these patients are limited and conflicting. Methods: We reviewed 110 MuSK‐MG patients from two large clinics in Italy and the USA. Results: Thirty‐nine to 49% of patients with generalized, acetylcholine receptor antibody (AChR‐Ab)–negative MG had MuSK‐MG. Eighty‐five percent were female, with disease onset typically in the fourth decade. Ocular and/or bulbar symptoms were present at onset in 79% of those studied. Eighty‐five percent were MGFA class III or greater, and crisis occurred in 28%. Plasma exchange (PLEX) produced improvement in 93%, whereas only 61% improved after intravenous immunoglobulin. Long‐term outcomes were comparable to those of patients with AChR‐Ab–positive MG. Conclusions: MuSK‐MG has a marked female predominance with frequent oculobulbar weakness and crises. Many patients deteriorate rapidly early in the disease, and PLEX is usually the preferred treatment. Long‐term outcomes are similar to those of patients with AChR‐Ab + MG. Muscle Nerve 44: 36–40, 2011