[系统性硬化症]
[Systemic sclerosis].
作者信息
Faber A-M, Krieg T, Hunzelmann N
机构信息
Klinik und Poliklinik für Dermatologie und Venerologie, Universität zu Köln, Kerpener Str. 62, 50937, Köln, Deutschland.
Translational Matrix Biology, Medizinische Fakultät, Universität zu Köln, Köln, Deutschland.
出版信息
Dermatologie (Heidelb). 2024 Mar;75(3):181-196. doi: 10.1007/s00105-024-05298-8. Epub 2024 Feb 16.
BACKGROUND
Systemic sclerosis (SSc) is a very heterogeneous, chronic, rare, but socioeconomically important disease with a severe disease course and severe impairment of the quality of life of affected patients.
OBJECTIVES
Overview of the current state of research on the pathogenesis, diagnosis and therapy of SSc.
METHODS
A literature search was performed.
RESULTS
The pathogenesis of SSc is not fully understood. ACR/EULAR criteria allow the diagnosis of early forms of SSc. Classification into limited cutaneous SSc and diffuse cutaneous SSc is of prognostic and therapeutic relevance. New organ-specific treatment options for SSc have led to improved quality of life and prognosis.
背景
系统性硬化症（SSc）是一种高度异质性的慢性罕见疾病，但在社会经济方面具有重要意义，其病程严重，会严重损害患者的生活质量。
目的
概述系统性硬化症发病机制、诊断和治疗的当前研究状况。
方法
进行了文献检索。
结果
系统性硬化症的发病机制尚未完全明确。美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）标准有助于诊断早期系统性硬化症。将其分为局限性皮肤型系统性硬化症和弥漫性皮肤型系统性硬化症具有预后和治疗意义。针对系统性硬化症的新的器官特异性治疗方案已改善了生活质量和预后。
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