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系统性硬皮病和硬斑病治疗的最新进展。

Recent Advances in Treatment of Systemic Sclerosis and Morphea.

机构信息

Department of Dermatology, Oregon Health and Science University, 3303 SW Bond Avenue, Portland, OR, 97239, USA.

出版信息

Am J Clin Dermatol. 2024 Mar;25(2):213-226. doi: 10.1007/s40257-023-00831-2. Epub 2023 Dec 12.

Abstract

Systemic sclerosis (SSc) and morphea are autoimmune sclerosing diseases that cause significant morbidity, and in the case of SSc, mortality. The pathogenesis of both SSc and morphea share vascular dysfunction, auto-reactive T cells and Th2-associated cytokines, such as interleukin 4, and overproduction of transforming growth factor beta (TGFβ). TGFβ stimulates fibroblast collagen and extra-cellular matrix production. Although morphea and SSc have similar pathogenic pathways and histological findings, they are distinct diseases. Recent advances in treatment of morphea, skin sclerosis in SSc, and interstitial lung disease in SSc are focused on targeting known pathogenic pathways.

摘要

系统性硬皮病(SSc)和硬斑病是自身免疫性硬化性疾病,会导致严重的发病率,在 SSc 的情况下还会导致死亡率。SSc 和硬斑病的发病机制都有血管功能障碍、自身反应性 T 细胞和 Th2 相关细胞因子,如白细胞介素 4,以及转化生长因子β(TGFβ)的过度产生。TGFβ刺激成纤维细胞胶原和细胞外基质的产生。尽管硬斑病和 SSc 具有相似的发病途径和组织学发现,但它们是不同的疾病。硬斑病、SSc 皮肤硬化和 SSc 间质性肺病治疗的最新进展侧重于针对已知的发病途径。

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