神経サルコイドーシスの新しい治療

New Treatment for Patients with Neurosarcoidosis

神経サルコイドーシスは組織診断が行いづらく確定診断が困難である。また症例数が少ないことから，治療薬のエビデンスに乏しい。他臓器サルコイドーシスと比較し神経サルコイドーシスは治療抵抗性のことが多い。治療の主軸はステロイドであるが，二次治療としてメトトレキサートなどの免疫抑制薬や抗腫瘍壊死因子α（TNF-α）製剤など，自他覚徴候や病勢により考慮していく。

Abstract

Diagnosis of possible neurosarcoidosis (NS) involves assessment of clinical presentation suggestive of NS and exclusion of other diagnoses, whereas its definitive diagnosis is challenging and it requires positive nervous system histology. Due to an exceptionally low incidence, limited data are available on the optimal therapeutic options for NS. NS is commonly associated with other sarcoidosis forms; however, the patients with NS experience more frequent relapses than with other organs sarcoidosis. While corticosteroid therapy is considered as the first-line of treatment for NS, secondary treatment options with immunosuppressive agents such as methotrexate, azathioprine, mycophenolate mofetil and tumor necrosis factor (TNF)-α inhibitors are considered based on the severity of NS manifestations and the disease status.