Guillain–Barré症候群：これからの治療

抄録

Guillain–Barré Syndrome (GBS) is an acute immune–mediated polyradiculoneuropathy where antibody–mediated complement activation plays an essential role. Randomized Clinical Trials (RCTs) have proved the efficacy of such immunomodulated therapy as plasmapheresis (PP) and intravenous immunoglobulin (IVIg). Outcome of GBS, however, is not as good as might be expected despite application of PP and IVIg. Thirty to forty percent of patients with GBS are intractable to PP or IVIg, 16% of GBS patients are unable to walk independently a year after the onset of GBS, and approximately 40% feel difficulty in walking or running 10 years after the onset of the disease, indicating that part of patients require additional treatments to improve the prognosis. As optional treatments, the second IVIg is considered for patients refractory to the first immunomodulating therapy, and IVIg with combination of intravenous methylprednisolone for patients in whom worse prognosis will be predicted, although the efficacy needs to be proved by RCTs. In addition to modified Erasmus GBS outcome score, delta IgG, and lower levels of serum albumin after IVIg, prognostic models to more precisely predict the clinical course and outcome at the early stage of the disease are required to provide better treatment. In view of the pathophysiology, a new treatment strategy will be developed. A phase 2 trial of eculizumab, a complement C5 inhibitor, was accomplished and it will be applied as an additional treatment in near future.