Rheumatology and Clinical Immunology Unit, Spedali Civili, Piazzale Spedali Civili 1, 25100 Brescia, Italy.
BMC Med. 2013 Apr 4;11:93. doi: 10.1186/1741-7015-11-93.
Sjögren's syndrome (SS) is a systemic progressive autoimmune disease characterized by a complex pathogenesis requiring a predisposing genetic background and involving immune cell activation and autoantibody production. The immune response is directed to the exocrine glands, causing the typical 'sicca syndrome', but major organ involvement is also often seen. The etiology of the disease is unknown. Infections could play a pivotal role: compared to normal subjects, patients with SS displayed higher titers of anti-Epstein-Barr virus (EBV) early antigens, but lower titers of other infectious agent antibodies such as rubella and cytomegalovirus (CMV) suggest that some infections may have a protective role against the development of autoimmune disease. Recent findings seem to show that low vitamin D levels in patients with SS could be associated with severe complications such as lymphoma and peripheral neuropathy. This could open new insights into the disease etiology. The current treatments for SS range from symptomatic therapies to systemic immunosuppressive drugs, especially B cell-targeted drugs in cases of organ involvement. Vitamin D supplementation may be an additional tool for optimization of SS treatment.
干燥综合征（SS）是一种系统性进行性自身免疫性疾病，其特征为发病机制复杂，需要有易患遗传背景，并涉及免疫细胞激活和自身抗体产生。免疫反应针对外分泌腺，导致典型的“干燥综合征”，但也常出现主要器官受累。该疾病的病因不明。感染可能起关键作用：与正常受试者相比，SS 患者的抗 Epstein-Barr 病毒（EBV）早期抗原滴度更高，但其他病原体抗体（如风疹和巨细胞病毒（CMV））滴度较低，提示某些感染可能对自身免疫性疾病的发展具有保护作用。最近的研究结果表明，SS 患者的维生素 D 水平低可能与淋巴瘤和周围神经病等严重并发症相关。这可能为疾病病因提供新的见解。SS 的当前治疗方法包括对症治疗和全身免疫抑制药物，特别是在有器官受累的情况下针对 B 细胞的药物。维生素 D 补充可能是 SS 治疗优化的另一种手段。
