Department of Rheumatology, University Hospital , Zurich , Switzerland.
Department of Medicine, Division of Rheumatology, David Geffen School of Medicine, University of California , Los Angeles , CA , USA.
Expert Rev Clin Immunol. 2019 Oct;15(10):1009-1017. doi: 10.1080/1744666X.2020.1668269. Epub 2019 Sep 30.
: Systemic sclerosis (SSc) is a rare and complex connective tissue disease characterized by fibrosis of the skin and internal organs. Interstitial lung disease (ILD) is a common complication of SSc and the leading cause of SSc-related death. No drugs are licensed for the treatment of SSc-ILD. : This review provides an overview of the current treatment of SSc-ILD and a perspective on investigational therapies, focusing on those studied in randomized controlled trials. : There is substantial room for improvement in the treatment of SSc-ILD. Current treatment focuses on immunosuppressant therapies, particularly cyclophosphamide and mycophenolate. Hematopoietic stem cell transplantation has been shown to improve long-term outcomes, but the risk of treatment-related mortality restricts its use to select patients at specialized centers. Modifying the course of disease to improve outcomes remains the goal for new therapies. Several drugs are under investigation as potential therapies for SSc-ILD, providing hope that the limited treatment armamentarium for SSc-ILD will be expanded and improved in the near future. Expert consensus is needed on how to screen for and monitor SSc-ILD and on when to initiate and escalate therapy.
系统性硬化症（SSc）是一种罕见且复杂的结缔组织疾病，其特征为皮肤和内脏器官纤维化。间质性肺疾病（ILD）是 SSc 的常见并发症，也是 SSc 相关死亡的主要原因。目前尚无药物获批用于治疗 SSc-ILD。
本文对 SSc-ILD 的现有治疗方法进行了综述，并对研究中的治疗方法进行了探讨，重点介绍了那些在随机对照试验中进行研究的治疗方法。
SSc-ILD 的治疗仍有很大的改进空间。目前的治疗方法主要集中在免疫抑制治疗上，特别是环磷酰胺和霉酚酸酯。造血干细胞移植已被证明可以改善长期预后，但治疗相关死亡率的风险限制了其在专门中心选择患者中的应用。改变疾病进程以改善预后仍然是新疗法的目标。几种药物正在作为 SSc-ILD 的潜在治疗方法进行研究，这为 SSc-ILD 的有限治疗方法在不久的将来得到扩展和改进带来了希望。需要就如何筛查和监测 SSc-ILD 以及何时开始和升级治疗达成专家共识。
