Immunotherapy in neuromuscular disorders

Isabel Illa MD (Dr. Illa of Hospital de la Santa Creu i Sant Pau has no relevant financial relationships to disclose.)
Jordi Diaz-Manera MD (Dr. Diaz-Manera of Hospital Santa Creu i Sant Pau in Barcelona, Spain, has received consulting fees from Genzyme.)
Luis Querol MD (Dr. Querol Gutierrez of Hospital de la Santa CreuiSant Pau in Barcelona, Spain, received a research grant from Novartis Spain and consulting fees from Bayer Pharma.)
Ricardo Rojas-Garcia MD PhD (Dr. Rojas-Garcia of Hospital de la Santa Creu i Sant Pau has received consulting fees from Pfizer.)
Salvatore DiMauro MD, editor. (Dr. DiMauro, Director Emeritus of H Houston Merritt Clinical Center for the Study of Muscular Dystrophy and Related Diseases at Columbia University, has no relevant financial relationships to disclose.)
Originally released September 8, 2011; last updated February 17, 2015; expires February 17, 2018

This article includes discussion of immunotherapy in neuromuscular disorders, chronic inflammatory polyradiculoneuropathy, multifocal motor neuropathy, paraproteinemic neuropathies, myasthenia gravis, and inflammatory myopathies. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.


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 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.

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