Ramírez Marta, Ramos-Casals Manuel, Graus Francesc
Laboratorio de Enfermedades Autoinmunes Josep Font, Servicio de Enfermedades Autoinmunes, IDIBAPS, Barcelona, Spain.
Med Clin (Barc). 2009 Sep 12;133(9):349-59. doi: 10.1016/j.medcli.2008.12.027. Epub 2009 Apr 19.
Sjögren syndrome (SS) is a systemic autoimmune disease that affects the exocrine glands and presents with sicca symptoms of the main mucosa surfaces. The spectrum of the disease extends from sicca syndrome to systemic involvement (extraglandular manifestations). Although earlier studies described central nervous system (CNS) involvement as a frequent extraglandular manifestation of primary SS, symptomatic CNS involvement is rarely described in recent large series (<5%). A wide spectrum of neurological features has been described, including not only asymptomatic white matter lesions in MR examinations, but also severe focal or diffuse neurological processes. Differential diagnosis includes the coexistence of other systemic autoimmune diseases (lupus, antiphospholipid syndrome, vasculitis), cerebrovascular disease and multiple sclerosis.
干燥综合征（SS）是一种影响外分泌腺的全身性自身免疫性疾病，主要黏膜表面会出现干燥症状。该疾病范围从干燥综合征到全身受累（腺体外表现）。尽管早期研究将中枢神经系统（CNS）受累描述为原发性SS常见的腺体外表现，但近期大型系列研究中很少描述有症状的CNS受累情况（<5%）。已描述了广泛的神经学特征，不仅包括磁共振检查中无症状的白质病变，还包括严重的局灶性或弥漫性神经病变。鉴别诊断包括其他全身性自身免疫性疾病（狼疮、抗磷脂综合征、血管炎）、脑血管疾病和多发性硬化症的共存。
