原发性干燥综合征（primary Sj-gren’s syndrome，pSS）是一类淋巴细胞浸润破坏外分泌腺的自身免疫性疾病，多起病隐匿，进展缓慢，系统受累以血液系统、肺间质病变较为多见，也可导致复杂多变的神经系统受累，如认知功能减退、脑膜炎、周围神经病变及视神经脊髓炎等。对于持续的自身免疫炎症中的血管损伤已逐渐受到关注，近年研究发现系统性红斑狼疮（systemic lupus erythematosus，SLE）患者易早期伴发动脉粥样硬化，除了传统危险因素影响外，与自身免疫炎症直接相关［1-2］。但对于pSS的缺血性脑卒中目前罕有报道，本研究报告1例以脑梗塞为主要表现的pSS中枢神经系统（central nervous system，CNS）受累的病例（以下简称CNS-pSS），并附相关文献复习，以探讨其发生和发展机制，提高医生对此类病变的关注。

Sj-gren’s syndrome (SS) is a chronic autoimmune disorder characterized by lymphocytes infiltration in the exocrine glands. Central nervous system complications of primary SS are not rare, but ischemic stroke has been rarely reported. Here we report a 43-year-old female with a two-year history of primary SS, presenting with sudden cerebral infarction. Her primary SS was diagnosed on the basis of clinical features, high levels of serum anti-SSA and anti-SSB antibodies, salivary gland secretion evaluation and positive sublingual gland biopsy results. Magnetic resonance imaging revealed infarct lesions in the parietal and occipital lobes, as well as in the left basal ganglia. Magnetic resonance angiography showed a remarkable stenosis in the left middle cerebral artery. Other differential diagnoses were ruled out. Corticosteroid and immunosuppressor, together with anti-platelet and statin were effective, and the patient recovered quickly without sequelae. Based on these findings, vasculitis due to primary SS should be considered among the causes of stroke. The literature was reviewed and the relationship between primary SS and cerebral infarction explored. The pathogenesis of ischemic stroke in primary SS is still unknown and warrants further studies.