Sorbonne Université, AP-HP, Hôpital Saint-Antoine, Service de Médecine Interne and Inflammation-Immunopathology-Biotherapy Department (DMU i3), Paris, France.
Hôpital Montfermeil, Service de Médecine Interne et Rhumatologie, Montfermeil, France.
Clin Exp Rheumatol. 2020 Jul-Aug;38 Suppl 126(4):103-109. Epub 2020 Oct 22.
Primary Sjögren's syndrome (pSS) is a common systemic autoimmune disease characterised by exocrinopathy resulting in dryness of the mouth and eyes, unexplained fatigue and diffuse pain. Neurological involvement is uncommon in pSS, involving the central nervous system in 2-5% of cases and more frequently the peripheral nervous system in 5-15% of cases. The diagnosis of pSS is to be considered when confronted with symptoms such as mouth and eye dryness, fatigue and pain, the most frequent of pSS symptoms. Objective measures of oral and eye dryness may help assert the diagnosis of pSS, as well as ACR/EULAR criteria. Differential diagnoses have to be excluded in patients exhibiting neurological symptoms, such as cryoglobulinaemic vasculitis or multiple sclerosis, before considering a neurological involvement specific to pSS. The treatment of these neurological manifestations takes into account different parameters, such as the presence of cryoglobulinaemic vasculitis, the severity of the symptoms, a rapidly progressing evolution and the failure of previous symptomatic treatments.
原发性干燥综合征（pSS）是一种常见的系统性自身免疫性疾病，其特征为外分泌腺病变导致口干、眼干、不明原因的疲劳和弥漫性疼痛。神经系统受累在 pSS 中并不常见，约 2-5%的病例累及中枢神经系统，5-15%的病例更常累及周围神经系统。当出现口干、眼干、疲劳和疼痛等 pSS 最常见的症状时，应考虑诊断为 pSS。客观的口腔和眼部干燥测量也有助于确定 pSS 的诊断，以及 ACR/EULAR 标准。在考虑与 pSS 相关的特定神经病变之前，必须排除出现神经系统症状的患者中的其他鉴别诊断，如冷球蛋白血症性血管炎或多发性硬化症。这些神经表现的治疗需要考虑到不同的参数，如冷球蛋白血症性血管炎的存在、症状的严重程度、快速进展的演变以及以前对症治疗的失败。
