系统性硬化症:2024 年回顾。

IF 3.4 4区 医学 Q2 RHEUMATOLOGY Clinical and experimental rheumatology Pub Date : 2024-08-01 Epub Date: 2024-07-26 DOI:10.55563/clinexprheumatol/is29he
Gemma Lepri, Marco Di Battista, Veronica Codullo, Francesco Bonomi, Antonello Sulis, Serena Guiducci, Alessandra Della Rossa
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引用次数: 0

摘要

系统性硬化症(SSc)是一种罕见的慢性结缔组织疾病,病因不明,主要有三个发病过程,即内皮损伤、炎症和免疫系统激活导致特异性自身抗体的产生以及最终的纤维化。SSc 是一种异质性疾病,将其分为两个亚组,即局限性皮肤亚组(lcSSc)和弥漫性皮肤亚组(dcSSc),并不能反映该病广泛而不同的表型表现。过去几年中,对 SSc 发病机制的认识、早期诊断和新治疗策略的提出都取得了进展,但 SSc 的治疗仍然是临床医生面临的一项挑战。因此,每年都有一些研究对疾病的发病机制、内脏器官受累情况和治疗方法提出新的见解。本综述旨在概述 2023 年发表的文献。
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Systemic sclerosis: one year in review 2024.

Systemic sclerosis (SSc) is a rare and chronic connective tissue disease of unknown aetiology and characterised by three main pathogenetic events represented by endothelial damage, inflammation with activation of the immune system leading to production of specific autoantibodies and finally fibrosis. SSc is a heterogeneous disease and the classification in two subsets, the limited cutaneous (lcSSc) subset and the diffuse cutaneous one (dcSSc), is not capable of capturing the broad and different phenotypic expression of the disease. In the last years progress has been made in the knowledge of SSc pathogenesis, in its early diagnosis and new therapeutic strategies have been proposed, however, the management of SSc still represents a challenge for the clinician. For this reason, every year several studies investigate new insights of disease pathogenesis, internal organ involvement and therapeutic approaches. The purpose of this review is to provide an overview of the literature published in 2023.

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来源期刊
CiteScore
6.10
自引率
18.90%
发文量
377
审稿时长
3-6 weeks
期刊介绍: Clinical and Experimental Rheumatology is a bi-monthly international peer-reviewed journal which has been covering all clinical, experimental and translational aspects of musculoskeletal, arthritic and connective tissue diseases since 1983.
期刊最新文献
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