ステロイド抵抗性の中枢神経サルコイドーシス—免疫抑制薬および生物学的製剤の使用

Steroid-Resistant Central Nervous System Sarcoidosis: Use of Immunosuppressants and Biologic Drugs

神経サルコイドーシスは不十分な治療や治療抵抗性により不可逆性の機能障害をきたす場合が少なくない。副腎皮質ステロイドが神経サルコイドーシスの第一選択薬であり，ステロイド抵抗性の場合には免疫抑制薬や腫瘍壊死因子α（TNF-α）阻害薬が用いられる。免疫抑制薬としてはメトトレキサートが頻用されており，TNF-α阻害薬としてはインフリキシマブの使用報告が多い。本論では上述の薬剤についてエビデンス，症例を踏まえて概説する。

Abstract

Sarcoidosis is a systemic non-caseating granulomatous disease of unknown origin, and involvement of the nervous system may result in irreversible neurological deficits. Corticosteroids (CSs) are commonly used as first-line agents for neurosarcoidosis. In steroid-refractory patients, immunosuppressants (ISs) have been used as second-line agents, and tumor necrosis factor-alpha (TNF-alpha) inhibitors as third-line agents. However, evidence regarding the treatment of steroid-refractory neurosarcoidosis is scarce, and treatment strategies for such patients have not been established. In this article, we review the evidence regarding treatments for neurosarcoidosis and strategies for refractory patients. We also discuss the practical uses of CS, IS, and TNF-α inhibitors, providing specific cases treated with such agents.