Fujisawa Miwako, Kanda Takashi
Department of Neurology, Yamaguchi Prefectural Grand Medical Center.
Brain Nerve. 2022 May;74(5):455-461. doi: 10.11477/mf.1416202064.
Sarcoidosis is a granulomatous multiorgan disease of unknown etiology that commonly affects the respiratory system, eyes, and skin, and less commonly affects the nervous system. Because of its rarity, a standard treatment for central nervous system (CNS) sarcoidosis has not yet been established. Corticosteroids remain the cornerstone of CNS sarcoidosis treatment. However, CNS sarcoidosis, other than isolated facial nerve paralysis, is often refractory to treatment and requires long-term corticosteroid treatment. In particular, patients with hydrocephalus have a high mortality rate and a lack of response to this treatment. Therefore, immunosuppressants, including TNF-α inhibitors and corticosteroids, should be considered as the initial treatment. For older patients, it is important to pay attention to infection as an adverse event and to the toxicity of the therapeutic agents. Because steroid-related adverse events are more common in the older patient group, the lowest effective dose should be used, and the treatment duration should be kept as short as possible after careful evaluation of disease activity. Corticosteroid-sparing agents are effective at reducing the cumulative toxicity of corticosteroids. Recently, various new potential agents have emerged and their efficacy has been assessed. It is expected that more treatment options will be available for CNS sarcoidosis in future.
结节病是一种病因不明的肉芽肿性多器官疾病,通常累及呼吸系统、眼睛和皮肤,较少累及神经系统。由于其罕见性,中枢神经系统(CNS)结节病的标准治疗方法尚未确立。皮质类固醇仍然是CNS结节病治疗的基石。然而,除孤立性面神经麻痹外,CNS结节病通常对治疗耐药,需要长期使用皮质类固醇治疗。特别是,脑积水患者死亡率高且对这种治疗无反应。因此,包括肿瘤坏死因子-α抑制剂和皮质类固醇在内的免疫抑制剂应被视为初始治疗方法。对于老年患者,重要的是要注意感染这一不良事件以及治疗药物的毒性。由于与类固醇相关的不良事件在老年患者组中更为常见,应使用最低有效剂量,并在仔细评估疾病活动后将治疗持续时间尽可能缩短。皮质类固醇节省剂在降低皮质类固醇的累积毒性方面有效。最近,出现了各种新的潜在药物并评估了它们的疗效。预计未来CNS结节病将有更多的治疗选择。