Sandhya Pulukool, Kurien Biji Theyilamannil, Danda Debashish, Scofield Robert Hal
825 NE 13th Street, Oklahoma City, OK 73104,. United States.
Curr Rheumatol Rev. 2017;13(1):5-22. doi: 10.2174/1573397112666160714164149.
Sjogren's syndrome is a common autoimmune disease that presents with sicca symptoms and extraglandular features. Sjogren's syndrome is presumably as common as RA; yet it is poorly understood, underdiagnosed and undertreated. From the usual identity as an autoimmune exocrinopathy to its most recent designate as an autoimmune epithelitis - the journey of SS is complex. We herein review some of the most important milestones that have shed light on different aspects of pathogenesis of this enigmatic disease. This includes role of salivary gland epithelial cells, and their interaction with cells of the innate and adaptive immune system. Non-immune factors acting in concert or in parallel with immune factors may also be important. The risk genes identified so far have only weak association, nevertheless advances in genetics have enhanced understanding of disease mechanisms. Role of epigenetic and environmental role factors is also being explored. SS has also some unique features such as congenital heart block and high incidence of lymphoma; disease mechanisms accounting for these manifestations are also reviewed.
干燥综合征是一种常见的自身免疫性疾病，表现为干燥症状和腺外特征。干燥综合征可能与类风湿关节炎一样常见；然而，人们对它了解甚少，诊断不足且治疗不充分。从其作为自身免疫性外分泌病的常见身份到最近被认定为自身免疫性上皮炎——干燥综合征的历程很复杂。我们在此回顾一些最重要的里程碑事件，这些事件揭示了这种神秘疾病发病机制的不同方面。这包括唾液腺上皮细胞的作用及其与固有免疫系统和适应性免疫系统细胞的相互作用。与免疫因素协同或并行起作用的非免疫因素也可能很重要。迄今为止确定的风险基因关联较弱，不过遗传学的进展增进了对疾病机制的理解。表观遗传学和环境因素的作用也在探索之中。干燥综合征还有一些独特特征，如先天性心脏传导阻滞和淋巴瘤的高发病率；本文也对解释这些表现的疾病机制进行了综述。
