导致系统性硬化症发病机制的衰老分子基础。

IF 5.2 2区 医学 Q1 RHEUMATOLOGY
Current opinion in rheumatology Pub Date : 2025-01-01 Epub Date: 2024-10-17 DOI:10.1097/BOR.0000000000001061
Monica M Yang, Francesco Boin, Paul J Wolters
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引用次数: 0

摘要

综述的目的:系统性硬化症(SSc)是一种以弥漫性器官纤维化和血管病变为特征的系统性自身免疫疾病。衰老异常与肺部和其他器官纤维化疾病的关系日益密切。本综述旨在总结衰老的既定机制,以及这些机制如何可能导致 SSc 的发病机制:最近的发现:患有间质性肺病(SSc-ILD)的SSc患者端粒缩短,并与疾病的严重程度和死亡率有关。虽然端粒长度缩短的原因不明,但免疫机制可能在其中起作用。衰老细胞积聚在 SSc 患者的受累器官中,形成了一种病理细胞表型,这种细胞表型可能是促纤维化和炎症性的。总结:包括端粒功能障碍和细胞衰老在内的衰老机制可能是导致SSc进行性纤维化、血管病变和免疫功能障碍的原因。要想了解异常衰老是疾病的诱因还是延续性因素,以及这是否具有细胞或器官特异性,还需要开展进一步的工作。更好地了解衰老机制在 SSc 中扮演的角色将有助于我们了解潜在的病理生物学,并可能影响对表现出衰老表型的患者的管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Molecular underpinnings of aging contributing to systemic sclerosis pathogenesis.

Purpose of review: Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by diffuse organ fibrosis and vasculopathy. Aberrant aging has been increasingly implicated in fibrotic diseases of the lung and other organs. The aim of this review is to summarize the established mechanisms of aging and how they may contribute to the pathogenesis of SSc.

Recent findings: Shortened telomeres are present in SSc patients with interstitial lung disease (SSc-ILD) and associate with disease severity and mortality. Although the cause of telomere length shortening is unknown, immune mechanisms may be at play. Senescent cells accumulate in affected organs of SSc patients and contribute to a pathologic cellular phenotype that can be profibrotic and inflammatory. In addition to identifying patients with a more severe phenotype, biomarkers of aging may help identify patients who have worse outcomes with immunosuppression.

Summary: Aging mechanisms, including telomere dysfunction and cellular senescence, likely contribute to the progressive fibrosis, vasculopathy, and immune dysfunction of SSc. Further work is needed to understand whether aberrant aging is an initiator or perpetuator of disease, and whether this is cell or organ specific. A better understanding of the role aging mechanisms play in SSc will contribute to our understanding of the underlying pathobiology and may also influence management of patients exhibiting the aging phenotype.

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来源期刊
Current opinion in rheumatology
Current opinion in rheumatology 医学-风湿病学
CiteScore
9.70
自引率
2.00%
发文量
89
审稿时长
6-12 weeks
期刊介绍: A high impact review journal which boasts an international readership, Current Opinion in Rheumatology offers a broad-based perspective on the most recent and exciting developments within the field of rheumatology. Published bimonthly, each issue features insightful editorials and high quality invited reviews covering two or three key disciplines which include vasculitis syndromes, medical physiology and rheumatic diseases, crystal deposition diseases and rheumatoid arthritis. Each discipline introduces world renowned guest editors to ensure the journal is at the forefront of knowledge development and delivers balanced, expert assessments of advances from the previous year.
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