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系统性硬皮病:2024 年回顾一年

Systemic sclerosis: one year in review 2024.

机构信息

Department of Experimental and Clinical Medicine, University of Florence, Section of Rheumatology, AOU Careggi, Florence, Italy.

Rheumatology Unit, University of Pisa, Italy.

出版信息

Clin Exp Rheumatol. 2024 Aug;42(8):1517-1528. doi: 10.55563/clinexprheumatol/is29he. Epub 2024 Jul 26.

Abstract

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.

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,其主要发病机制有三个,分别是内皮损伤、免疫系统激活导致特定自身抗体产生的炎症,以及最终的纤维化。SSc 是一种异质性疾病,将其分为局限性皮肤型(lcSSc)和弥漫性皮肤型(dcSSc)两种亚型,无法完全反映出疾病的广泛和不同的表型表现。近年来,人们对 SSc 的发病机制、早期诊断以及新的治疗策略有了更多的了解,但 SSc 的治疗仍然是临床医生面临的一个挑战。因此,每年都有多项研究探讨疾病发病机制、内脏器官受累和治疗方法的新见解。本文的目的是对 2023 年发表的文献进行综述。

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