Fibrosis and Immune Dysregulation in Systemic Sclerosis
Fibrosis and Immune Dysregulation in Systemic Sclerosis
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DOI：
10.1007/978-3-319-98143-7_2
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发表时间：
2018-11
期刊：
影响因子：
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通讯作者：
Y. Argobi;G. Smith
中科院分区：
文献类型：
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作者：
Y. Argobi;G. Smith
系统性硬化症是一种罕见的结缔组织疾病，病因不明，以皮肤和内脏硬化为特征。它有一个复杂的发病机制，包括血管异常和伴随的自身免疫导致组织硬化。本章综述了目前对系统性硬化症发病机制的认识，综述了免疫失衡的作用及其与血管损伤和持续性硬化症的关系。系统性硬化症与其他结缔组织疾病不同，仅靠免疫抑制药物通常不足以控制疾病，而开发其他方面的治疗方法，即血管病变和纤维化，对系统性硬化症至关重要。在过去的十年中，对动物和人类的研究揭示了有关系统性硬化症分子机制的宝贵信息。此外，已经有一些针对系统性硬化症中涉及的特定细胞因子和信号通路的临床试验，这些试验有助于排除潜在的治疗途径。将这些不同研究的发现结合起来，应该有助于缩小寻找新疗法的范围。
Systemic sclerosis is a rare connective tissue disease of unclear etiology characterized by cutaneous and internal organ sclerosis. It has a complex pathogenesis believed to include vascular abnormalities with accompanying autoimmunity leading to tissue sclerosis. This chapter summarizes the current knowledge about systemic sclerosis pathogenesis and reviews the role of immune imbalance and its relationship with vascular damage and sustained sclerosis. Systemic sclerosis is unlike other connective tissue diseases in that immunosuppressive drugs alone are usually insufficient to control the disease and developing treatments for the other aspects, i.e., vasculopathy and fibrosis, is paramount in systemic sclerosis. Over the past decade, studies on animals and humans have revealed valuable information about systemic sclerosis molecular mechanisms. In addition, there have been a number of clinical trials targeting specific cytokines and signaling pathways involved in systemic sclerosis that have helped eliminate potential therapeutic pathways from consideration. Combining the findings from these disparate studies should help narrow the search for novel therapies.