Section of Rheumatology and allergy, Yale School of medicine, Yale University, New Haven, CT, USA.
Department of Biosciences, Durham University, Stockton Road, Durham, UK. steven.o'
Curr Rheumatol Rep. 2020 Jun 19;22(8):42. doi: 10.1007/s11926-020-00918-3.
Systemic sclerosis (SSc) is an autoimmune connective tissue disease in which there is an activation of fibroblast to a myofibroblast that secretes huge amounts of extracellular matrix. Currently, no treatment exists that modifies the fibrosis elements and new therapeutic targets are badly needed. This review examines the current state of treatments and emerging therapeutics.
Nintedanib was found to significantly reduce the rate of decline in SSc associated FVC, although it has no benefit on skin fibrosis. New cannabinoid receptor2 agonist has shown superb effects in phase II and results in phase III are anticipated. Other targets are currently being tested in clinical trials and new targets that are yet to be tested are increasing in the SSc literature. Nintedanib is now licenced for SSc interstitial lung disease but this does not modify the skin fibrosis. Current ongoing trials will determine the role of various targets. New targets are emerging as we gain a deeper understanding of disease pathogenesis.
系统性硬化症(SSc)是一种自身免疫性结缔组织疾病,其中成纤维细胞被激活为肌成纤维细胞,分泌大量细胞外基质。目前尚无改变纤维化成分的治疗方法,迫切需要新的治疗靶点。这篇综述探讨了目前的治疗方法和新出现的治疗方法。
尼达尼布被发现可显著降低 SSc 相关 FVC 的下降速度,尽管它对皮肤纤维化没有益处。新型大麻素受体 2 激动剂在 II 期研究中显示出优异的效果,预计 III 期研究结果将会公布。其他靶点目前正在临床试验中进行测试,SSc 文献中不断出现新的有待测试的靶点。尼达尼布目前被许可用于治疗 SSc 间质性肺病,但不能改变皮肤纤维化。目前正在进行的试验将确定各种靶点的作用。随着我们对疾病发病机制的深入了解,新的靶点不断涌现。