神経内科における静脈内免疫グロブリンの使用

In the past 3 decades, intravenous immunoglobulin (IVIg) - pooled IgG (IgG) obtained from the plasma of several thousands of healthy donors - has had a major impact in the successful treatment of previously poorly controlled autoimmune neurologic disorders. Based on randomized controlled trials, IVIg is approved for the treatment of Guillain-Barré syndrome, chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN), and dermatomyositis. It has been effective in myasthenia gravis exacerbations and stiff-person syndrome, and exhibits convincing efficacy in autoimmune encephalitides. As maintenance therapy, IVIg and subcutaneous IgG (SCIg) are effective in controlled studies only in CIDP and MMN. The demand for IVIg therapy is ever-growing, resulting in supply shortages, leading to strategies to replace IVIg with recombinant products based on proposed mechanisms that confer its anti-inflammatory activity. This chapter illustrates clinical applications of IVIg in neurologic diseases, discusses the merit of SCIg, and addresses practically important issues, such as the need for dose adjustment, cycling intervals, dependency testing, or treatment discontinuation.