Baldini Chiara, Chatzis Loukas G, Fulvio Giovanni, La Rocca Gaetano, Pontarini Elena, Bombardieri Michele
Rheumatology Unit, University of Pisa, Italy.
Pathophysiology Department, School of Medicine, National and Kapodistrian University of Athens, Greece.
Clin Exp Rheumatol. 2024 Dec;42(12):2336-2343. doi: 10.55563/clinexprheumatol/i8iszc. Epub 2024 Dec 10.
The pathogenesis of Sjögren's disease (SjD) is still elusive; however, the disease is widely recognised as a multistep disorder triggered by the interplay of environmental, hormonal and genetic factors. Innate immune system plays a crucial role in the initiation of the inflammatory process, but the amplification and the perpetuation of the autoimmune process require a continual interaction between the innate and adaptive immune systems. Several important contributions elucidating SjD pathogenesis have been recently published due to emerging technologies. This review provides an overview of the recent literature focusing, in the first part, on new insights into genetic and epigenetics studies. In the second part, we will discuss new findings related to salivary epithelial glandular cells and their interaction with other immune cells, type I interferon signature and innate immunity. Finally, as ectopic germinal centres like structures in the salivary glands of patients with SjD have been critically involved in autoreactive B cell activation and have been associated with progression towards B cell lymphomas, we will focus on new insights into their regulation in SjD and novel insights into the transition to lymphoma. Hopefully, a better comprehension of SjD complexity will pave the way to highly targeted therapeutic strategies.
干燥综合征(SjD)的发病机制仍不明确;然而,该疾病被广泛认为是一种由环境、激素和遗传因素相互作用引发的多步骤疾病。固有免疫系统在炎症过程的启动中起关键作用,但自身免疫过程的放大和持续需要固有免疫系统与适应性免疫系统之间持续的相互作用。由于新兴技术的出现,最近发表了几篇阐明SjD发病机制的重要论文。本综述概述了近期文献,第一部分聚焦于遗传和表观遗传学研究的新见解。第二部分,我们将讨论与唾液腺上皮细胞及其与其他免疫细胞相互作用、I型干扰素特征和固有免疫相关的新发现。最后,由于干燥综合征患者唾液腺中类似异位生发中心的结构在自身反应性B细胞激活中起关键作用,并与向B细胞淋巴瘤的进展相关,我们将聚焦于干燥综合征中其调节的新见解以及向淋巴瘤转变的新见解。希望对干燥综合征复杂性的更好理解将为高度靶向的治疗策略铺平道路。