Immunotherapy in neuromuscular disorders

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By Isabel Illa MD PhD, Jordi Diaz-Manera MD, Luis Querol MD, and Ricardo Rojas-Garcia MD PhD

An increasing number of immunotherapies are being used to treat the heterogeneous group of neuromuscular diseases believed to have an autoimmune pathogenesis. Therapeutic algorithms based on current knowledge are detailed for the most frequent of these diseases: Inflammatory neuropathies, myasthenia gravis and the inflammatory myopathies. New therapies used in these diseases, including biological agents (monoclonal antibodies or recombinant proteins), are also reviewed.

Key points:

  • Classic immunosuppressants remain the most beneficial and widely used drugs for immune-mediated neuromuscular diseases.
  • The majority (60% to 80%) of patients with immune-mediated neuromuscular diseases improve and have a good quality of life if they receive the appropriate drug, at the appropriate dose, for the appropriate time.
  • Several new specific therapies developed recently are potential treatments for the immune-mediated neuromuscular diseases provided we improve our understanding of the cells and pathways involved in the pathogenesis of the disorders.
  • Rituximab emerges as a therapeutic option for myasthenia gravis patients resistant to other therapies, especially those with MuSK+MG.
  • Multifocal motor neuropathy patients usually improve with intravenous immunoglobulin but not with any of the other immunosuppressants, a phenomenon not fully understood.