旨在评估中枢神经系统 (CNS) 神经结节病 (NS) 的脑脊液 (CSF) 细胞因子/趋化因子谱及其在鉴别诊断、治疗和预后中的效用。在这项病例对照研究中，我们验证了 17 种细胞因子/趋化因子（白细胞介素 [IL]-1-β、IL-2、IL-4、IL-5、IL-6、IL-10、IL-12p70、IL-13、IL-17A、BAFF、IL-8/CXCL8、CXCL9 、CXCL10、CXCL13、GM-CSF、干扰素-γ 和肿瘤坏死因子 [TNF]-α）在多重自动免疫分析系统（ELLA；Bio-Techne，明尼阿波利斯，明尼苏达州，美国）中进行评估，并在脑脊液和血清中对其进行评估有症状的可能或明确的 CNS NS (01/2011-02/2023) 患者，伴有钆增强和/或脑脊液细胞增多。患有多发性硬化症、原发性中枢神经系统淋巴瘤、水通道蛋白 4 免疫球蛋白 G 阳性、非炎症性疾病和健康个体的患者被用作对照。总共 32 名 NS 患者（59% 为女性；中位年龄 59 岁 [19-81] ) 被包括在内； 12 名患者获得了同期血清。脑脊液对照包括 26 名多发性硬化症患者、8 名原发性 CNS 淋巴瘤患者、84 名水通道蛋白 4 免疫球蛋白 G 阳性患者和 34 名非炎症性疾病患者。 32 名 NS 患者中有 31 名出现钆增强，32 名患者中有 27 名 (84%) 出现脑脊液细胞增多。 NS 患者的 CSF IL-2、IL-6、IL-10、IL-13、BAFF、IL-8/CXCL8、CXCL9、CXCL10、CXCL13、GM-CSF、干扰素-γ 和 TNF-α 水平显着升高与非炎症对照相比（p ≤ 0.02）；脑脊液中的升高比血清中更常见。 32 名 NS 患者中有 18 名同时出现 IL-6、CXCL9、CXCL10、GM-CSF、干扰素-γ 和 TNF-α 升高，但仅在 1 名对照中出现。 IL-6、IL-10、IL-13、CXCL9、CXL10、GM-CSF 和 TNF-α 升高与疾病活动度相关。NS CSF 细胞因子/趋化因子谱表明 T 细胞（主要是 T 辅助细胞类型 1）、巨噬细胞和 B 细胞参与。这些特征有助于 NS 诊断、指示疾病活动并提出治疗途径。 ANN NEUROL 2024。© 2024 美国神经病学协会。

To evaluate the cerebrospinal fluid (CSF) cytokine/chemokine profile of central nervous system (CNS) neurosarcoidosis (NS), and its utility in differential diagnosis, treatment, and prognostication.In this case-control study, we validated 17 cytokines/chemokines (interleukin [IL]-1-beta, IL-2, IL-4, IL-5, IL-6, IL-10, IL-12p70, IL-13, IL-17A, BAFF, IL-8/CXCL8, CXCL9, CXCL10, CXCL13, GM-CSF, interferon-gamma, and tumor necrosis factor [TNF]-alpha) in a multiplexed automated immunoassay system (ELLA; Bio-Techne, Minneapolis, MN, USA), and assessed them in CSF and serum of symptomatic patients with probable or definite CNS NS (01/2011-02/2023) with gadolinium enhancement and/or CSF pleocytosis. Patients with multiple sclerosis, primary CNS lymphoma, aquaporin-4 immunoglobulin G positivity, non-inflammatory disorders, and healthy individuals were used as controls.A total of 32 NS patients (59% women; median age, 59 years [19-81]) were included; concurrent sera were available in 12. CSF controls consisted of 26 multiple sclerosis, 8 primary CNS lymphoma, 84 aquaporin-4 immunoglobulin G positive, and 34 patients with non-inflammatory disorders. Gadolinium enhancement was present in 31 of 32 NS patients, and CSF pleocytosis in 27 of 32 (84%). CSF IL-2, IL-6, IL-10, IL-13, BAFF, IL-8/CXCL8, CXCL9, CXCL10, CXCL13, GM-CSF, interferon-gamma, and TNF-alpha levels were significantly higher in NS patients compared with non-inflammatory controls (p ≤ 0.02); elevations were more common in CSF than serum. Concurrent elevation of IL-6, CXCL9, CXCL10, GM-CSF, interferon-gamma, and TNF-alpha was present in 18 of 32 NS patients, but only in 1 control. Elevated IL-6, IL-10, IL-13, CXCL9, CXL10, GM-CSF, and TNF-alpha associated with measures of disease activity.NS CSF cytokine/chemokine profiles suggest T cell (mainly T helper cell type 1), macrophage, and B-cell involvement. These signatures aid in NS diagnosis, indicate disease activity, and suggest therapeutic avenues. ANN NEUROL 2024.© 2024 American Neurological Association.