High efficacy of rituximab for myasthenia gravis: a comprehensive nationwide study in Austria

J Neurol. 2019 Mar;266(3):699-706. doi: 10.1007/s00415-019-09191-6. Epub 2019 Jan 16.


Background: Most patients with myasthenia gravis (MG) need long-term immunosuppressive therapy. However, conventional agents may have intolerable side effects, take too long or fail to achieve disease control. Rituximab (RTX) has emerged as an off-label treatment for refractory MG, but data on its use are still sparse.

Methods: We conducted a retrospective nationwide study contacting all Austrian neurologists to provide anonymized data of all adult MG patients treated with RTX and minimum follow-up of 3 months. The Myasthenia Gravis Foundation of America Postintervention Status scale was used to assess outcomes.

Results: 34 (60.7%) of a total of 56 patients were women. Median (IQR) age at diagnosis of MG and start of RTX were 41.5 (24.3; 65.8) and 47.5 (33; 71) years, respectively. Antibodies (ab) against acetylcholine receptor (AchR) and muscle-specific tyrosine kinase (MuSK) were present in 69.6% and 25% of patients, respectively (seronegative: 5.4%). Before RTX, 47 (83.9%) patients had had plasma exchange, immune adsorption or immunoglobulins. Three months after RTX, 14 of 53 (26.4%) patients were in remission. At last follow-up after a median of 20 (10; 53) months, remission was present in 42.9% of patients and another 25% had minimal manifestations. Remission was more frequent in patients with MuSK ab vs. those with AchR ab (71.4% vs. 35.9%, p = 0.022). RTX was safe. The presence of MuSK ab independently predicted remission after RTX.

Conclusion: In this retrospective study on RTX for MG, the largest to date, RTX appeared safe, efficacious and fast acting. Benefit from RTX was greatest in MuSK ab + MG.

Keywords: Anti-MuSK; Efficacy; Myasthenia gravis; Outcome; Rituximab.

MeSH terms

  • Adult
  • Aged
  • Austria
  • Cohort Studies
  • Dose-Response Relationship, Drug
  • Female
  • Humans
  • Immunologic Factors / therapeutic use*
  • Logistic Models
  • Male
  • Middle Aged
  • Myasthenia Gravis / drug therapy*
  • Receptor Protein-Tyrosine Kinases / immunology
  • Receptors, Cholinergic / immunology
  • Rituximab / therapeutic use*
  • Treatment Outcome*


  • Immunologic Factors
  • Receptors, Cholinergic
  • Rituximab
  • MUSK protein, human
  • Receptor Protein-Tyrosine Kinases