Pers J-O, Le Pottier L, Devauchelle V, Saraux A, Youinou P
Laboratoire d'immunologie, centre hospitalier universitaire de Brest, B.P. 824, 29609 Brest, France.
Rev Med Interne. 2008 Dec;29(12):1000-6. doi: 10.1016/j.revmed.2008.01.018. Epub 2008 Apr 10.
Sjögren's syndrome (SS) is an autoimmune epithelitis hallmarked by a disruption of epithelial cells, the subsequent lymphocytic infiltration of lachrymal and salivary glands (SGs), and their ensuing dryness. One may posit that SS is triggered by viruses, and/or modulated by sex steroid hormones, and there is indeed a consensus that its aetiology is multifactorial, with genetic factors interacting with environmental agents.
T-cells have long occupied central stage of the debate on the type of lymphocytes involved in the pathogenesis of SS. The relevance of B cells has, however, been emphasized over the past five years and new insights into their functions revealed. Furthermore, increased levels of the B-cell activating factor (BAFF) may be responsible for quantitative and qualitative anomalies of B-cells found in SS such as emergence of self reactive B-cells. This review reports compelling evidence that B-cells are involved in the pathophysiology of SS.
Since SS may thus be conceived as a model for B-cell-induced autoimmunity, it is no surprise that B-cell ablative-treatment has proven to be relatively effective in SS.
干燥综合征(SS)是一种自身免疫性上皮炎,其特征为上皮细胞破坏、随后泪腺和唾液腺(SGs)出现淋巴细胞浸润以及随之而来的腺体干燥。有人认为SS由病毒引发,和/或受性类固醇激素调节,并且人们普遍认为其病因是多因素的,遗传因素与环境因素相互作用。
在关于参与SS发病机制的淋巴细胞类型的争论中,T细胞长期占据核心地位。然而,在过去五年中,B细胞的相关性得到了强调,并且对其功能有了新的认识。此外,B细胞活化因子(BAFF)水平升高可能是SS中发现的B细胞数量和质量异常的原因,例如自身反应性B细胞的出现。本综述报告了令人信服的证据,表明B细胞参与了SS的病理生理学。
由于SS可被视为B细胞诱导的自身免疫的模型,因此毫不奇怪,B细胞消融治疗在SS中已被证明相对有效。