Long-lasting treatment effect of rituximab in MuSK myasthenia

Neurology. 2012 Jan 17;78(3):189-93. doi: 10.1212/WNL.0b013e3182407982. Epub 2012 Jan 4.


Objective: Rituximab has emerged as an efficacious option for drug-resistant myasthenia gravis (MG). However, reports published only describe the short-term follow-up of patients treated and little is known about their long-term clinical and immunologic evolution. Our objective was to report the clinical and immunologic long-term follow-up of 17 patients (6 MuSK+MG and 11 AChR+MG) and compare the response between AChR+MG and MuSK+MG patients.

Methods: Myasthenia Gravis Foundation America postintervention status and changes in treatment and antibody titers were periodically determined. Lymphocyte subpopulations, total immunoglobulin, immunoglobulin G (IgG) anti-MuSK subclasses, and anti-tetanus toxoid IgG before and after treatment were also studied.

Results: After a mean post-treatment period of 31 months, 10 of the AChR+MG patients improved but 6 of them needed reinfusions. In contrast, all MuSK+MG patients achieved a remission (4/6) or minimal manifestations (2/6) status and no reinfusions were needed. Consequently, in the MuSK+MG group, prednisone doses were significantly reduced and concomitant immunosuppressants could be withdrawn. Clinical improvement was associated with a significant decrease in the antibody titers only in the 6 MuSK+MG patients. At last follow-up MuSK antibodies were negative in 3 of these patients and showed a decrease of over 80% in the other 3.

Conclusion: In view of the long-lasting benefit observed in MuSK+MG patients, we recommend to use rituximab as an early therapeutic option in this group of patients with MG if they do not respond to prednisone.

Classification of evidence: This study provides Class IV evidence that IV rituximab improves the clinical and immunologic status of patients with MuSK+MG.

Publication types

  • Clinical Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antibodies, Monoclonal, Murine-Derived / therapeutic use*
  • Autoantibodies / blood*
  • Female
  • Humans
  • Immunologic Factors / therapeutic use
  • Longitudinal Studies
  • Male
  • Myasthenia Gravis / blood*
  • Myasthenia Gravis / diagnosis
  • Myasthenia Gravis / drug therapy*
  • Receptor Protein-Tyrosine Kinases / immunology*
  • Receptors, Cholinergic / immunology*
  • Rituximab
  • Treatment Outcome


  • Antibodies, Monoclonal, Murine-Derived
  • Autoantibodies
  • Immunologic Factors
  • Receptors, Cholinergic
  • Rituximab
  • MUSK protein, human
  • Receptor Protein-Tyrosine Kinases