自己免疫性脳炎の治療の進歩

抄録

The current treatment of autoimmune encephalitis primarily involves immunotherapy and, in paraneoplastic cases, tumor–directed therapy. Immunotherapy typically involves intravenous steroid pulse therapy, intravenous immunoglobulin, and plasma exchange during the acute phase. In severe cases, additional treatments such as rituximab or cyclophosphamide are often administered. For recurrent cases, oral corticosteroids and immunosuppressive agents such as azathioprine are commonly used as maintenance therapy. Before initiating immunotherapy, it is essential to screen for latent infections and hematologic abnormalities such as cytopenia. After starting treatment, regular monitoring of liver function, renal function, and blood cell counts is necessary to ensure safety and detect adverse effects early. These approaches are largely based on expert opinion rather than robust clinical evidence, and many are not covered by national health insurance systems. To address this gap, several randomized, double–blind, placebo–controlled trials are currently underway, investigating the efficacy and safety of agents such as inebilizumab, satralizumab, rozanolixizumab, and bortezomib in patients with autoimmune encephalitis. Conventional therapies often rely on broad immunosuppression or non–selective antibody removal, which may result in suboptimal efficacy and frequent adverse effects. Consequently, there is growing interest in the development of more targeted and less toxic therapeutic strategies. One such promising approach is chimeric antigen receptor (CAR) T–cell therapy, which offers the potential for highly specific immune modulation with reduced systemic side effects.