北京大学学报(医学版) ›› 2016, Vol. 48 ›› Issue (6): 1077-1080. doi: 10.3969/j.issn.1671-167X.2016.06.027

• 病例报告 • 上一篇    下一篇

原发干燥综合征伴发脑梗塞1例报告附文献复习

杨丹1,乔琳2,赵丽丹2△   

  1. (1. 中国医学科学院,北京协和医学院,国家心血管病中心,心血管疾病国家重点实验室,阜外心血管病医院心内科, 北京100037;2. 中国医学科学院,北京协和医学院,北京协和医院风湿免疫科, 北京100730)
  • 出版日期:2016-12-18 发布日期:2016-12-18
  • 通讯作者: 赵丽丹 E-mail:zhaolidan@hotmail.com

Cerebral infarction in a patient with primary Sj-gren’s syndrome: a case report and literature review

YANG Dan1, QIAO Lin2, ZHAO Li-dan2△   

  1. (1. Department of Cardiology, Fuwai Hospital, State Key Laboratory of Cardiovascular Disease, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China; 2.Department of Rheumatology & Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China)
  • Online:2016-12-18 Published:2016-12-18
  • Contact: ZHAO Li-dan E-mail:zhaolidan@hotmail.com

摘要:

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。

关键词: 干燥综合征, 脑梗塞, 中枢神经系统, 糖皮质激素类

Abstract:

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.

Key words: Sj-gren’s syndrome, Brain infarction, Central nervous system, Glucocorticoids

中图分类号: 

  • R593.2
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