Department of Rheumatology, Shimane University Faculty of Medicine, Shimane, Japan.
Scand J Immunol. 2012 Jun;75(6):633-40. doi: 10.1111/j.1365-3083.2012.02694.x.
Neuro-Behçet's disease (NBD) is a serious complication of Behçet's disease. Generally, NBD patients with a chronic course are refractory to immunosuppressive treatment, resulting in the deterioration of personality. In this study, levels of B cell-activating factor belonging to the TNF family (BAFF) were measured in the cerebrospinal fluid (CSF) from 18 patients with NBD, 27 patients with epidemic aseptic meningitis (AM), 24 patients with multiple sclerosis (MS) and 34 healthy controls. BAFF levels in patients with NBD were significantly elevated compared with healthy controls, but showed no statistically significant elevation compared with either of the disease controls. In contrast, CSF IL-6 levels were slightly elevated in patients with NBD and significantly elevated in patients with AM and MS compared with healthy controls. Patients with NBD were subdivided into two groups according to their clinical course (eight patients with a slowly progressive course presenting with psychosis and dementia and 10 patients with an acute course including aseptic meningitis, brainstem involvement and myelopathy). BAFF levels were significantly increased in those with a slowly progressive course compared with those with an acute course. CSF BAFF levels did not correlate with serum BAFF levels, CSF cell counts or CSF IL-6 levels in patients with NBD. These data suggested that BAFF was produced within the central nervous system and may be associated with the development of NBD, particularly with a progressive course.
神经白塞病（NBD）是白塞病的严重并发症。一般来说，NBD 患者病程呈慢性，对免疫抑制治疗反应差，导致人格恶化。在这项研究中，我们测量了 18 例 NBD 患者、27 例流行性无菌性脑膜炎（AM）患者、24 例多发性硬化症（MS）患者和 34 例健康对照者的脑脊液（CSF）中 B 细胞激活因子属于 TNF 家族（BAFF）的水平。与健康对照组相比，NBD 患者的 BAFF 水平显著升高，但与任何疾病对照组相比均无统计学意义上的升高。相比之下，NBD 患者的 CSF IL-6 水平略有升高，而 AM 和 MS 患者则明显高于健康对照组。根据临床病程，将 NBD 患者分为两组（8 例缓慢进展型患者表现为精神病和痴呆，10 例急性型患者包括无菌性脑膜炎、脑干受累和脊髓病变）。与急性病程患者相比，缓慢进展型患者的 BAFF 水平显著升高。NBD 患者的 CSF BAFF 水平与血清 BAFF 水平、CSF 细胞计数或 CSF IL-6 水平均无相关性。这些数据表明，BAFF 是在中枢神经系统内产生的，可能与 NBD 的发生，特别是与进展性病程有关。
