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干燥综合征:流行病学、分类标准、分子发病机制、诊断与治疗

Sjögren's Syndrome: Epidemiology, Classification Criteria, Molecular Pathogenesis, Diagnosis, and Treatment.

作者信息

Hu Ying, Wen Bin, Zhang Yunfei, Wang Xiaocui, Duan Xuemei, Li Haonan, Fan Yufeng, Shang Huifeng, Jing Yukai

机构信息

Department of Clinical Laboratory, Third Hospital of Shanxi Medical University, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences Tongji Shanxi Hospital Taiyuan China.

出版信息

MedComm (2020). 2025 Jul 11;6(7):e70297. doi: 10.1002/mco2.70297. eCollection 2025 Jul.


DOI:10.1002/mco2.70297
PMID:40656544
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12246557/
Abstract

Sjögren's syndrome (SS) is a chronic autoimmune disorder characterized by T-cell-mediated B-cell hyperactivity and cytokine production, clinically manifesting, dry mouth and eyes, accompanied by pain and fatigue. The disease may progress from asymptomatic glandular involvement to systemic manifestations or even lymphoma. The pathogenesis of SS is intricate, involving a multifaceted interplay of genetic, environmental, and immunological factors. There is still uncertainty regarding the effectiveness of SS-targeted treatments, due to the significant diversity in disease phenotypes and potentially varying responses to immunomodulatory therapies, stringent enrollment criteria and adoption of outcome metrics in clinical trials may partially explain the failure of many trials to achieve their primary outcomes. Despite the current lack of effective treatments, recent advancements have been made in epidemiology, the development of classification criteria, and the establishment of systems for assessing disease activity. Notably, enhanced insights into the pathogenesis have paved the way for the potential development of targeted therapies. This review aims to systematically synthesize the latest research advancements in the epidemiological characteristics, diagnostic criteria, molecular mechanisms, and clinical manifestations of SS, thereby providing a scientific foundation for the development of future therapeutic strategies.

摘要

干燥综合征(SS)是一种慢性自身免疫性疾病,其特征为T细胞介导的B细胞过度活跃和细胞因子产生,临床表现为口干、眼干,并伴有疼痛和疲劳。该疾病可能从无症状的腺体受累发展为全身表现,甚至淋巴瘤。干燥综合征的发病机制错综复杂,涉及遗传、环境和免疫因素的多方面相互作用。由于疾病表型存在显著差异,以及对免疫调节疗法的反应可能各不相同,针对干燥综合征的治疗效果仍存在不确定性,临床试验中严格的入选标准和采用的疗效指标可能部分解释了许多试验未能达到其主要结局的原因。尽管目前缺乏有效的治疗方法,但在流行病学、分类标准的制定以及疾病活动评估系统的建立方面取得了最新进展。值得注意的是,对发病机制的深入了解为靶向治疗的潜在发展铺平了道路。本综述旨在系统总结干燥综合征在流行病学特征、诊断标准、分子机制和临床表现方面的最新研究进展,从而为未来治疗策略的制定提供科学依据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/b88c3bc5edfa/MCO2-6-e70297-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/5692086a8d49/MCO2-6-e70297-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/dfb7117d96db/MCO2-6-e70297-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/ff7fafdf94fd/MCO2-6-e70297-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/08cd447e2269/MCO2-6-e70297-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/9aaac4088aef/MCO2-6-e70297-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/b88c3bc5edfa/MCO2-6-e70297-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/5692086a8d49/MCO2-6-e70297-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/dfb7117d96db/MCO2-6-e70297-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/ff7fafdf94fd/MCO2-6-e70297-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/08cd447e2269/MCO2-6-e70297-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/9aaac4088aef/MCO2-6-e70297-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0669/12246557/b88c3bc5edfa/MCO2-6-e70297-g006.jpg

相似文献

[1]
Sjögren's Syndrome: Epidemiology, Classification Criteria, Molecular Pathogenesis, Diagnosis, and Treatment.

MedComm (2020). 2025-7-11

[2]
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[4]
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[5]
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[8]
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[9]
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Clin Gastroenterol Hepatol. 2025-5-19

[10]
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本文引用的文献

[1]
An overview on Sjögren's syndrome and systemic lupus erythematosus' genetics.

Toxicol Res (Camb). 2025-2-23

[2]
Hydroxychloroquine: A double‑edged sword (Review).

Mol Med Rep. 2025-4

[3]
The Role of the Toll-Like Receptor Signaling Pathway in Autoimmune Diseases and Treatment with Traditional Chinese Medicine: A Literature Review.

Endocr Metab Immune Disord Drug Targets. 2025-1-21

[4]
Safety and efficacy of CAR-T cell therapy in patients with autoimmune diseases: a systematic review.

Rheumatol Int. 2025-1-4

[5]
The advent of chimeric antigen receptor T Cell therapy in recalibrating immune balance for rheumatic autoimmune disease treatment.

Front Pharmacol. 2024-12-12

[6]
Uncovering the Therapeutic Target and Molecular Mechanism of Upadacitinib on Sjogren's Syndrome.

Biomed Eng Comput Biol. 2024-10-23

[7]
Sjogren's syndrome: Everything you always wanted to know about genetic and epigenetic factors.

Autoimmun Rev. 2024-12

[8]
Loss of STIM1 and STIM2 in Salivary Glands Disrupts ANO1 Function but Does Not Induce Sjogren's Disease.

Function (Oxf). 2025-2-12

[9]
CAR immunotherapy in autoimmune diseases: promises and challenges.

Front Immunol. 2024

[10]
Nipocalimab in Early-Onset Severe Hemolytic Disease of the Fetus and Newborn.

N Engl J Med. 2024-8-8

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