Johnson Sindhu R, van den Hoogen Frank, Devakandan Keshini, Matucci-Cerinic Marco, Pope Janet E
Toronto Scleroderma Program, Department of Medicine, Mount Sinai Hospital, Toronto Western Hospital, Toronto, Ontario, Canada.
Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada.
Eur J Rheumatol. 2020 Oct;7(Suppl 3):S222-S227. doi: 10.5152/eurjrheum.2020.19116. Epub 2020 Jul 6.
Systemic sclerosis (SSc) is a heterogeneous disease with variability in autoantibody profiles, skin and internal organ involvement, disease trajectory, and survival. The ability to identify more homogeneous subsets of SSc patients has informed patient care and been an essential aspect of SSc research. In this article, the historic evolution of subsetting systems in SSc are described including clinically based SSc subsetting systems, their utility, strengths, and limitations. There is a shifting paradigm of SSc subsets, including biologic classification of SSc subsets and fully data-driven approaches to SSc subset classification, taking into consideration the needs of the SSc global community in the modern era and the ability to prognosticate patients with SSc.
系统性硬化症（SSc）是一种异质性疾病，在自身抗体谱、皮肤和内脏器官受累情况、疾病进程及生存率方面存在差异。识别系统性硬化症患者更具同质性亚组的能力为患者护理提供了依据，并且一直是系统性硬化症研究的重要方面。在本文中，我们描述了系统性硬化症亚组划分系统的历史演变，包括基于临床的系统性硬化症亚组划分系统、其效用、优势及局限性。系统性硬化症亚组划分的模式正在转变，包括系统性硬化症亚组的生物学分类以及完全基于数据驱动的系统性硬化症亚组分类方法，同时考虑到现代全球系统性硬化症患者群体的需求以及对系统性硬化症患者进行预后评估的能力。
