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干燥综合征:病因、病理生理学及潜在治疗干预综述

Sjogren's syndrome: Review of the aetiology, Pathophysiology & Potential therapeutic interventions.

作者信息

Nair Jisha J, Singh Tejas P

机构信息

College of Medicine and Dentistry, James Cook University, Townsville, Australia.

出版信息

J Clin Exp Dent. 2017 Apr 1;9(4):e584-e589. doi: 10.4317/jced.53605. eCollection 2017 Apr.

DOI:10.4317/jced.53605
PMID:28469828
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5410683/
Abstract

BACKGROUND

Sjogren's syndrome (SS) is an autoimmune disorder characterised by lymphocytic infiltration of exocrine glands, resulting in glandular dysfunction. Objectives: This study aims to review the aetiology of Sjogren's syndrome, highlight aspects that contribute to the pathophysiology of the disease and explore treatment options that target different mediators of pathogenesis.

MATERIAL AND METHODS

The MEDLINE/PubMed and Google Scholar databases were searched systematically with the terms "Sjogren's syndrome"; "clinical"; "treatment"; "management". Eligible studies had to meet a predefined inclusion criteria.

RESULTS

912 identified studies were evaluated against the inclusion criteria. 25 eligible studies were included for review. Sjogren's syndrome is a multifactorial condition with genetic, environmental and hormonal factors playing a role in establishing the condition. B-cell activating factor (BAFF) is an important mediator in the induction and perpetuation of this condition. Elevated BAFF levels, found in patients with SS, promote growth of B-cells and subsequent production of autoantibody; anti-SSA/Ro. BAFF inhibitors are important potential therapeutic drugs that may be effective in patients with Sjogren's syndrome. Other potential targets include CD20 and CD22 that cause B-cell depletion.

CONCLUSIONS

The pathophysiology of this exocrinopathy has not fully been elucidated. Potential therapeutic interventions include BAFF inhibitors and anti-CD20 and anti-CD22 therapy. However, no clinical trials have been conducted on subjects with Sjogren's syndrome to support existing research. Sjogren's syndrome, autoimmune, rheumatology.

摘要

背景

干燥综合征(SS)是一种自身免疫性疾病,其特征是外分泌腺淋巴细胞浸润,导致腺体功能障碍。目的:本研究旨在综述干燥综合征的病因,强调导致该疾病病理生理学的各个方面,并探索针对发病机制中不同介质的治疗选择。

材料与方法

使用“干燥综合征”“临床”“治疗”“管理”等术语系统检索MEDLINE/PubMed和谷歌学术数据库。符合条件的研究必须满足预先定义的纳入标准。

结果

根据纳入标准对912项已识别的研究进行了评估。纳入25项符合条件的研究进行综述。干燥综合征是一种多因素疾病,遗传、环境和激素因素在发病过程中起作用。B细胞活化因子(BAFF)是该疾病诱导和持续存在的重要介质。在干燥综合征患者中发现的BAFF水平升高,促进B细胞生长并随后产生自身抗体;抗SSA/Ro。BAFF抑制剂是可能对干燥综合征患者有效的重要潜在治疗药物。其他潜在靶点包括导致B细胞耗竭的CD20和CD22。

结论

这种外分泌腺病的病理生理学尚未完全阐明。潜在的治疗干预措施包括BAFF抑制剂以及抗CD20和抗CD22治疗。然而,尚未对干燥综合征患者进行临床试验以支持现有研究。干燥综合征,自身免疫,风湿病学。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6e9/5410683/4cd180f7c547/jced-9-e584-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6e9/5410683/4cd180f7c547/jced-9-e584-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d6e9/5410683/4cd180f7c547/jced-9-e584-g001.jpg

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Anti-Ro52 autoantibodies from patients with Sjögren's syndrome inhibit the Ro52 E3 ligase activity by blocking the E3/E2 interface.干燥综合征患者的抗 Ro52 自身抗体通过阻断 E3/E2 界面抑制 Ro52 E3 连接酶活性。
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