Department Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, P.O. Box 30.001, 9700 RB Groningen, The Netherlands.
J Autoimmun. 2012 Aug;39(1-2):103-11. doi: 10.1016/j.jaut.2012.01.009. Epub 2012 Feb 15.
Primary Sjögren's syndrome (pSS) is an autoimmune exocrinopathy characterized by chronic inflammation and destruction of the salivary and lacrimal glands. B- and T- lymphocyte infiltrations in the salivary glands with development of germinal center-like structures are characteristic for pSS. Overexpression of soluble factors, such as interferon α (IFNα) and B-cell activating factor (BAFF), are supposed to be important factors in the initiation and continuation of this disorder. The efficacy and success of B-cell depleting therapy in reducing disease activity in pSS patients for about six to nine months supports the notion that B-cells are major key players in disease manifestation of pSS. In addition to B-cells, also Th-cells (mainly Th17) seem to be involved in the pathogenetic process. In this review, we will discuss recent research findings regarding the cytokines IFNα and BAFF as wells as the role of B- and T-cells in pSS. Emphasis will be put on the impact of B-cell depletion therapy as well as on the presumed impact of therapies aimed for targeting BAFF, either as a sole modality or as a combined treatment with B-cell depletion.
原发性干燥综合征（pSS）是一种自身免疫性外分泌疾病，其特征为慢性炎症和唾液腺及泪腺的破坏。B 细胞和 T 细胞在唾液腺中的浸润伴有生发中心样结构的形成是 pSS 的特征。可溶性因子的过度表达，如干扰素 α（IFNα）和 B 细胞激活因子（BAFF），被认为是这种疾病发生和持续的重要因素。B 细胞耗竭疗法在大约 6 至 9 个月内降低 pSS 患者的疾病活动度的疗效和成功支持了 B 细胞是 pSS 疾病表现的主要关键因素的观点。除了 B 细胞外，Th 细胞（主要是 Th17）似乎也参与了发病过程。在这篇综述中，我们将讨论最近关于细胞因子 IFNα和 BAFF 以及 B 细胞和 T 细胞在 pSS 中的作用的研究结果。重点将放在 B 细胞耗竭疗法的影响上，以及针对 BAFF 的治疗方法（单独或与 B 细胞耗竭联合治疗）的假定影响上。
