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干燥综合征的发病机制:2024年回顾

Pathogenesis of Sjögren's disease: one year in review 2024.

作者信息

Baldini Chiara, Chatzis Loukas G, Fulvio Giovanni, La Rocca Gaetano, Pontarini Elena, Bombardieri Michele

机构信息

Rheumatology Unit, University of Pisa, Italy.

Pathophysiology Department, School of Medicine, National and Kapodistrian University of Athens, Greece.

出版信息

Clin Exp Rheumatol. 2024 Dec;42(12):2336-2343. doi: 10.55563/clinexprheumatol/i8iszc. Epub 2024 Dec 10.

DOI:10.55563/clinexprheumatol/i8iszc
PMID:39656593
Abstract

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.

摘要

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。

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1
Pathogenesis of Sjögren's disease: one year in review 2024.干燥综合征的发病机制:2024年回顾
Clin Exp Rheumatol. 2024 Dec;42(12):2336-2343. doi: 10.55563/clinexprheumatol/i8iszc. Epub 2024 Dec 10.
2
Immune and non-immune mediators in the fibrosis pathogenesis of salivary gland in Sjögren's syndrome.干燥综合征唾液腺纤维化发病机制中的免疫和非免疫介质。
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[Clonal B-cell reaction in Sjögren disease and Hashimoto autoimmune thyroiditis].[干燥综合征和桥本自身免疫性甲状腺炎中的克隆性B细胞反应]
Pathologe. 1996 Jul;17(4):289-95. doi: 10.1007/s002920050167.
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MicroRNA in Sjögren's Syndrome: Their Potential Roles in Pathogenesis and Diagnosis.干燥综合征中的 microRNA:其在发病机制和诊断中的潜在作用。
J Immunol Res. 2018 Jun 7;2018:7510174. doi: 10.1155/2018/7510174. eCollection 2018.
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Aspects of innate immunity in Sjögren's syndrome.干燥综合征的固有免疫相关问题。
Arthritis Res Ther. 2011 May 27;13(3):218. doi: 10.1186/ar3318.
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Salivary Gland Pathology in Sjögren's Syndrome.干燥综合征的唾液腺病理学
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Current views on the pathogenesis of Sjögren's syndrome.目前对干燥综合征发病机制的看法。
Curr Opin Rheumatol. 2018 Mar;30(2):215-221. doi: 10.1097/BOR.0000000000000473.
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Age-associated B cell infiltration in salivary glands represents a hallmark of Sjögren's-like disease in aging mice.年龄相关的 B 细胞浸润唾液腺代表了老年小鼠类干燥综合征的一个标志。
Geroscience. 2024 Dec;46(6):6085-6099. doi: 10.1007/s11357-024-01159-3. Epub 2024 Apr 24.
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Advances in cellular and molecular pathways of salivary gland damage in Sjögren's syndrome.干燥综合征唾液腺损伤的细胞和分子途径的研究进展。
Front Immunol. 2024 Jul 10;15:1405126. doi: 10.3389/fimmu.2024.1405126. eCollection 2024.

引用本文的文献

1
Identification of key interactive genes and their potential biological functions in type 2 diabetes and Sjögren's syndrome.2型糖尿病和干燥综合征中关键相互作用基因的鉴定及其潜在生物学功能
Sci Rep. 2025 Aug 6;15(1):28660. doi: 10.1038/s41598-025-14044-6.
2
Clinical Implications of Antinuclear Antibody (ANA) and Anti-Ro/Anti-La Antibody Profile in Patients with Primary Sjögren's Syndrome: A Multicenter Cross-Sectional Study of a National Egyptian Cohort.原发性干燥综合征患者抗核抗体(ANA)和抗Ro/抗La抗体谱的临床意义:埃及全国队列的多中心横断面研究
Open Access Rheumatol. 2025 Jul 14;17:135-146. doi: 10.2147/OARRR.S515325. eCollection 2025.
3
Exosomes as immunomodulators in autoimmune inflammation: implications for primary Sjögren's disease.
外泌体作为自身免疫性炎症中的免疫调节剂:对原发性干燥综合征的意义。
Inflamm Res. 2025 Jun 13;74(1):91. doi: 10.1007/s00011-025-02053-0.