Sjogren syndrome
Sjogren syndrome
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DOI：
10.1038/nrdp.2016.47
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发表时间：
2016-07-07
影响因子：
81.5
通讯作者：
Ramos-Casals, Manuel
中科院分区：
文献类型：
--
作者：
Brito-Zeron, Pilar;Baldini, Chiara;Ramos-Casals, Manuel
干燥综合征（SjS）是一种全身性自身免疫性疾病，主要影响外分泌腺（主要是唾液腺和泪腺），并导致粘膜表面（主要是口腔和眼睛）严重干燥。这种疾病主要影响中年妇女，但也可以在儿童，男性和老年人中观察到。SjS的临床表现是异质性的，可以从干燥症状到全身性疾病（特征在于受影响组织的上皮周淋巴细胞浸润或免疫复合物沉积）和淋巴瘤变化。SjS发展的潜在机制是外分泌腺上皮的破坏，这是对自身抗原Ro/SSA和La/SS B等的异常B细胞和T细胞应答的结果。SjS的诊断标准包括患者血清中自身抗体的检测和活检唾液腺组织的组织学分析。SjS的治疗方法包括局部和全身治疗，以控制疾病的干燥和全身症状。SjS是一种严重的疾病，死亡率过高，主要与疾病的全身受累和部分患者发生淋巴瘤有关。对SjS的认识已经有了很大的进展，但这种疾病的特征仍然是干燥症症状，疾病的全身受累，淋巴细胞浸润到外分泌腺，抗Ro/SSA和抗La/SSB自身抗体的存在，以及SjS患者淋巴瘤风险的增加。
Sjogren syndrome (SjS) is a systemic autoimmune disease that primarily affects the exocrine glands (mainly the salivary and lacrimal glands) and results in the severe dryness of mucosal surfaces, principally in the mouth and eyes. This disease predominantly affects middle-aged women, but can also be observed in children, men and the elderly. The clinical presentation of SjS is heterogeneous and can vary from sicca symptoms to systemic disease (characterized by peri-epithelial lymphocytic infiltration of the affected tissue or the deposition of the immune complex) and lymphoma. The mechanism underlying the development of SjS is the destruction of the epithelium of the exocrine glands, as a consequence of abnormal B cell and T cell responses to the autoantigens Ro/SSA and La/SSB, among others. Diagnostic criteria for SjS include the detection of autoantibodies in patient serum and histological analysis of biopsied salivary gland tissue. Therapeutic approaches for SjS include both topical and systemic treatments to manage the sicca and systemic symptoms of disease. SjS is a serious disease with excess mortality, mainly related to the systemic involvement of disease and the development of lymphomas in some patients. Knowledge of SjS has progressed substantially, but this disease is still characterized by sicca symptoms, the systemic involvement of disease, lymphocytic infiltration to exocrine glands, the presence of anti-Ro/SSA and anti-La/SSB autoantibodies and the increased risk of lymphoma in patients with SjS.