Rodrigues Ana Raquel, Soares Raquel
a Department of Biochemistry , Faculty of Medicine, University of Porto , Porto , Portugal and.
b Instituto de Investigação e Inovação em Saúde da Universidade do Porto , Porto , Portugal.
Autoimmunity. 2017 May;50(3):141-150. doi: 10.1080/08916934.2017.1280027. Epub 2017 Feb 14.
Sjögren's syndrome (SS) is an autoimmune disease most commonly characterized by ocular and oral dryness. Despite the high prevalence of SS, generation and perpetuation of this disease is still unclear in many aspects. Inflammation, nonetheless, seems to play a central role in this pathology especially in the form of Th-1, Th-2 and Th-17 cytokines release within different aspects, concentrations and connections involved in the maintenance of the syndrome. Moreover, the chronically created pro-inflammatory environment appears to promote glandular atrophy and irreparable architectural modifications. The establishment of germinal centers (GC) in SS are considered the main reason for the 16-times increased probability of lymphoproliferative disease development in these patients. SS is also interconnected with numerous others auto-inflammatory and autoimmune diseases, many of them representing the first clear sign of a dysregulated immune system. Despite the recent advances, treatment options are still insufficient. This overview aims to better elucidate this local and systemic disease, which can be of vital use not only for SS but also to other rheumatic pathological conditions.
干燥综合征(SS)是一种自身免疫性疾病,最常见的特征是眼干和口干。尽管干燥综合征患病率很高,但在许多方面,这种疾病的发生和持续存在仍不清楚。然而,炎症似乎在这种病理过程中起着核心作用,特别是以Th-1、Th-2和Th-17细胞因子在维持该综合征所涉及的不同方面、浓度和联系中释放的形式。此外,长期形成的促炎环境似乎会促进腺体萎缩和不可修复的结构改变。干燥综合征中生发中心(GC)的形成被认为是这些患者淋巴增殖性疾病发生概率增加16倍的主要原因。干燥综合征还与许多其他自身炎症性和自身免疫性疾病相互关联,其中许多是免疫系统失调的首个明显迹象。尽管最近取得了进展,但治疗选择仍然不足。本综述旨在更好地阐明这种局部和全身性疾病,这不仅对干燥综合征至关重要,而且对其他风湿性病理状况也具有重要意义。