Department of Neurology, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku-gun, Ishikawa, 920-0293, Japan.
Department of Hematology and Immunology, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku-gun, Ishikawa, 920-0293, Japan.
J Neuroinflammation. 2018 May 18;15(1):151. doi: 10.1186/s12974-018-1196-3.
Rheumatoid meningitis (RM) is a rare disorder that often develops during a remission phase of rheumatoid arthritis (RA). This is the first study to demonstrate differences in regard to immunological disturbance between blood and cerebrospinal fluid (CSF) samples obtained from a patient with RM using flow cytometry.
A 36-year-old woman with RA and generalized myasthenia gravis (MG) developed RM during a remission phase. Although both RA and MG were stable and well controlled, she noticed fever, headache, and transient sensory disturbance. Blood and CSF examination findings suggested aseptic meningitis, while brain magnetic resonance imaging revealed restricted portions of meningitis and associated cortical lesions, compatible with a diagnosis of RM. The dose of oral prednisolone was increased, which ameliorated the symptoms within 1 week along with improvement in CSF findings. This patient exhibited features of RM that were manifested in a manner independent of the activity of RA. An investigation of cellular immunity using CSF specimens with flow cytometry showed differences in regard to the pathogenesis of inflammation in the CSF as compared to outside of the central nervous system. In contrast to results obtained with paired blood samples, CSF cells at the peak stage of RM showed a marked increase in CCR3 Th2 cells and marked decrease in CD8 cells, suggesting an immunoregulatory disturbance in the CSF. Those findings indicated a CSF-specific activation of humoral immunity, resulting in augmentation of meningeal inflammation, as shown by excess synthesis of intrathecal IgG and markedly elevated interleukin-6 level. Results of the present detailed investigation of lymphocyte subsets revealed a discrepancy regarding the process of inflammation in this RM patient between CSF and blood samples.
RM is not a simple reflection of the immune status of RA, as the pathogenesis seems related to, at least in part, CSF-specific immunological dysregulation.
类风湿性脑膜炎（RM）是一种罕见的疾病，通常在类风湿性关节炎（RA）缓解期发生。这是第一项使用流式细胞术研究 RM 患者血液和脑脊液（CSF）样本免疫紊乱差异的研究。
一名 36 岁女性，患有 RA 和全身性重症肌无力（MG），在缓解期发生 RM。尽管 RA 和 MG 均稳定且得到良好控制，但她出现发热、头痛和短暂感觉障碍。血液和 CSF 检查结果提示无菌性脑膜炎，而脑磁共振成像显示脑膜炎和相关皮质病变受限，符合 RM 诊断。增加口服泼尼松龙剂量，1 周内症状改善，CSF 检查结果改善。该患者表现出 RM 的特征，与 RA 的活动无关。使用流式细胞术对 CSF 标本进行细胞免疫研究表明，与中枢神经系统外相比，CSF 中的炎症发病机制存在差异。与配对血样结果相比，RM 高峰时 CSF 细胞中 CCR3 Th2 细胞明显增加，CD8 细胞明显减少，提示 CSF 中存在免疫调节紊乱。这些发现表明 CSF 特异性激活体液免疫，导致脑膜炎症加剧，表现为鞘内 IgG 过度合成和白细胞介素-6 水平明显升高。对淋巴细胞亚群的详细研究结果表明，该 RM 患者 CSF 和血液样本之间的炎症过程存在差异。
RM 不是 RA 免疫状态的简单反映，其发病机制似乎与至少部分 CSF 特异性免疫失调有关。
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