Department of Allergy and Rheumatology, Nippon Medical School , Tokyo, Japan.
Expert Rev Clin Immunol. 2020 Jun;16(6):547-560. doi: 10.1080/1744666X.2020.1777857. Epub 2020 Jun 17.
Interstitial lung disease (ILD) is a leading cause of death in patients with systemic sclerosis (SSc). Nonspecific immunosuppressants have been the first-line treatment for SSc-associated ILD (SSc-ILD). Nintedanib, an oral triple kinase inhibitor targeting profibrotic pathways, has been employed for the treatment of idiopathic pulmonary fibrosis and has recently received marketing approval in the United States and Japan, based on the results of a placebo-controlled randomized controlled trial. In this clinical trial, nintedanib delayed the progression of SSc-ILD compared with placebo.
This review covers current pharmacotherapies for SSc-ILD, drug profiles of nintedanib, and efficacy and safety profiles of nintedanib in patients with idiopathic pulmonary fibrosis and SSc-ILD observed in randomized controlled trails.
Currently, we have two treatment options for SSc-ILD, i.e., immunosuppressants and antifibrotic agents. However, appropriate utilization of antifibrotic agents in clinical practice remains challenging, i.e., in which cases they are to be used, timing of use, how to use them properly, and whether in combination with immunosuppressants.
间质性肺病(ILD)是系统性硬化症(SSc)患者死亡的主要原因。非特异性免疫抑制剂一直是治疗系统性硬化症相关间质性肺病(SSc-ILD)的一线药物。尼达尼布是一种针对成纤维细胞增生途径的口服三激酶抑制剂,已被用于治疗特发性肺纤维化,最近基于安慰剂对照随机临床试验的结果,在美国和日本获得了上市批准。在这项临床试验中,尼达尼布与安慰剂相比,延缓了 SSc-ILD 的进展。
本综述涵盖了 SSc-ILD 的当前药物治疗方法、尼达尼布的药物特征,以及在特发性肺纤维化和 SSc-ILD 患者中观察到的尼达尼布的疗效和安全性。
目前,我们有两种治疗 SSc-ILD 的选择,即免疫抑制剂和抗纤维化药物。然而,在临床实践中适当利用抗纤维化药物仍然具有挑战性,例如,在哪些情况下使用它们、使用时机、如何正确使用以及是否与免疫抑制剂联合使用。