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参与干燥综合征发病机制、诊断和治疗的免疫因子。

The immune factors involved in the pathogenesis, diagnosis, and treatment of Sjogren's syndrome.

作者信息

Huang Yi-fan, Cheng Qian, Jiang Chun-miao, An Shu, Xiao Lan, Gou Yong-chao, Yu Wen-jing, Lei Lei, Chen Qian-ming, Wang Yating, Wang Jun

机构信息

State Key Laboratory of Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China.

出版信息

Clin Dev Immunol. 2013;2013:160491. doi: 10.1155/2013/160491. Epub 2013 Jul 9.

Abstract

Sjogren's syndrome (SS) is a systemic, autoimmune disorder characterized by salivary insufficiency and lymphocytic infiltration of the exocrine glands. Even though the mechanism of its pathology and progression has been researched ever since its discovery, the roles of different parts of immune system remain inconclusive. There is no straightforward and simple theory for the pathogenesis and diagnosis of Sjogren's syndrome because of the multiple kinds and functions of autoantibodies, changing proportion of different T-lymphocyte subsets with the progression of disease, unsuspected abilities of B lymphocytes discovered recently, crosstalk between cytokines connecting the factors mentioned previously, and genetic predisposition that contributes to the initiation of this disease. On the other hand, the number of significant reports and open-label studies of B-cell depletion therapy showing clinical efficacy in sjogren's syndrome has continued to accumulate, which provides a promising future for the patients. In a word, further elucidation of the role of different components of the immune system will open avenues for better diagnosis and treatment of SS, whose current management is still mainly supportive.

摘要

干燥综合征(SS)是一种全身性自身免疫性疾病,其特征为唾液分泌不足和外分泌腺淋巴细胞浸润。尽管自发现以来对其病理机制和进展进行了研究,但免疫系统不同部分的作用仍不明确。由于自身抗体的种类和功能繁多、不同T淋巴细胞亚群比例随疾病进展而变化、近期发现的B淋巴细胞的意外功能、连接上述因素的细胞因子之间的相互作用以及导致该疾病发生的遗传易感性,干燥综合征的发病机制和诊断尚无直接简单的理论。另一方面,关于B细胞耗竭疗法在干燥综合征中显示临床疗效的重要报告和开放标签研究的数量不断积累,这为患者带来了充满希望的未来。总之,进一步阐明免疫系统不同成分的作用将为更好地诊断和治疗干燥综合征开辟道路,目前干燥综合征的治疗仍以支持治疗为主。

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