Division of Rheumatology, Department of Internal Medicine, University of Texas Health Science Center at Houston, McGovern Medical School, Houston, Texas, USA.
The first Affiliated Hospital, Guangxi University of Science and Technology, Liuzhou, Guangxi, China.
Curr Opin Rheumatol. 2023 Nov 1;35(6):364-370. doi: 10.1097/BOR.0000000000000967. Epub 2023 Aug 21.
Systemic sclerosis (SSc) is a multisystem autoimmune connective tissue disease characterized by early inflammation followed by excessive fibrosis in the skin and internal organs. Enhancing our comprehension of SSc pathogenesis is essential to develop effective therapeutic strategies. Animal models that mimic one or more aspects of SSc have been proven to be a valuable resource for investigating disease mechanisms. This review aims to provide an updated overview of the existing SSc animal models and the potentially relevant pathways to SSc pathogenesis.
This review focuses on the most recently generated and investigated animal models, which delve into novel pathways beyond existing models or employ genetic technologies to gain a deeper understanding of SSc pathogenesis including activation of early type I interferon (IFN) signaling pathway, immune cell function and pulmonary artery hypertension (PAH).
While no single animal model can fully replicate SSc, a combination of different models can offer valuable insights into the pathways involved in the onset and advancement of the SSc. These insights can prove animal models as a crutial preclinical tool for developing effective treatments for SSc.
系统性硬化症(SSc)是一种多系统自身免疫性结缔组织病,其特征为早期炎症,随后皮肤和内脏器官出现过度纤维化。深入了解 SSc 的发病机制对于开发有效的治疗策略至关重要。模拟 SSc 一个或多个方面的动物模型已被证明是研究疾病机制的宝贵资源。本综述旨在提供现有 SSc 动物模型的最新概述,以及与 SSc 发病机制相关的潜在途径。
本综述重点介绍了最近生成和研究的动物模型,这些模型深入探讨了现有模型之外的新途径,或利用遗传技术深入了解 SSc 的发病机制,包括早期 I 型干扰素(IFN)信号通路的激活、免疫细胞功能和肺动脉高压(PAH)。
虽然没有单一的动物模型可以完全复制 SSc,但不同模型的组合可以为 SSc 发病和进展中涉及的途径提供有价值的见解。这些见解可以证明动物模型是开发 SSc 有效治疗方法的重要临床前工具。