Berglová Irena, Krejsek Jan, Kolácková Martina, Slezák Radovan
Department of Clinical Immunology and Allergology, Charles University in Prague, Faculty of Medicine, Czech Republic.
Acta Medica (Hradec Kralove). 2011;54(2):51-7. doi: 10.14712/18059694.2016.18.
Sjögren's syndrome (SS) is a chronic autoimmune immunopathological disease of unknown aetiology. It is characterized by focal lymphocyte infiltration and inflammation in exocrinne glands, involving especially salivary and lacrimal glands. Hypofunction of the glands leads to the decreased glandular secretion together with impaired production of saliva and tears, resulting in dryness of the mouth and eyes (xerostomia and xerophthalmia, respectively). Some of the studies have suggested that Toll-like receptors and B cells play a pivotal role in the pathogenesis of autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis and SS etc. Stimulation of B cells via the TLRs pathway leads to several important changes including increase in antibody production, differentiation to plasma cells, cytokine production and up-regulation of molecules essential for antigen presentation to (autoreactive) T cells. Experimental data support the idea that co-engagement of BCR and TLR might be sufficient for B cell activation and lead to the failure of tolerance. In human naive B cells, most TLRs are expressed at very low or undetectable level, but expression of TLR 7 and 9 is rapidly induced by B cell receptor triggering. This review will focus on the possible role of B cells and TLRs signaling in the pathogenesis of SS.
干燥综合征(SS)是一种病因不明的慢性自身免疫性免疫病理疾病。其特征是外分泌腺出现局灶性淋巴细胞浸润和炎症,尤其累及唾液腺和泪腺。腺体功能减退导致腺体分泌减少,同时唾液和泪液分泌受损,从而导致口干和眼干(分别为口干燥症和干眼病)。一些研究表明,Toll样受体和B细胞在系统性红斑狼疮、类风湿关节炎和干燥综合征等自身免疫性疾病的发病机制中起关键作用。通过Toll样受体途径刺激B细胞会导致多种重要变化,包括抗体产生增加、分化为浆细胞、细胞因子产生以及对抗原呈递给(自身反应性)T细胞所必需分子的上调。实验数据支持这样的观点,即BCR和Toll样受体的共同作用可能足以激活B细胞并导致免疫耐受失败。在人类幼稚B细胞中,大多数Toll样受体表达水平极低或无法检测到,但B细胞受体触发可迅速诱导Toll样受体7和9的表达。本综述将重点探讨B细胞和Toll样受体信号传导在干燥综合征发病机制中的可能作用。