2020 Volume 37 Issue 4 Pages 558-561
In immune–mediated peripheral neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP) and Guillain–Barré syndrome (GBS), intravenous immunoglobulin therapy (IVIg), plasmapheresis therapy, and corticosteroids therapy had been established by the 1990s and have achieved a certain success. However, there is a need for more effective treatment of long–term neurological decline in severe GBS cases or CIDP cases with recurrent exacerbations. In addition, for MAG antibody–associated neuropathy, there is no treatment with sufficiently established efficacy. In response to these treatment demands, new treatment approaches have recently been put into practical use, or movements toward practical use in the future have been progressing. These movements can be broadly divided into the following three categories : (1) modifying existing treatment methods to improve the quality of treatment as a whole, including patient quality of life ; (2) expanding of indications of existing medicines, (3) new therapeutic approaches. As (1), the indication for maintenance IVIg in CIDP has been introduced, and the administration time has been shortened by high–concentration γ–globulin preparation, and self–injection (subcutaneous injection) at home has also become possible. As (2), the long–term prognosis improving effect of GBS of eculizumab which is an anti–complement drug was reported. There is also a demand for expanding the coverage of rituximab for MAG antibody–positive neuropathy. As (3), degradation of immunoglobulin degrading enzymes (IdeS : IgG degrading enzymes of Streptococcus pyogenes) in GBS and synthetic HNK–1 sugar chain epitope polymers for MAG antibody–associated neuropathy have been under development. In addition to these, there have been reports of the application of biomarkers such as anti–neurofascin155 antibody and anti–contactin–1 antibody in CIDP for treatment selection.
