Introduction

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.

Areas covered

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.

Expert opinion

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.

中文翻译：

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尼达尼布用于治疗系统性硬化症相关的间质性肺病。

简介

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已成为 SSc 相关 ILD (SSc-ILD) 的一线治疗方法。尼达尼布是一种靶向促纤维化途径的口服三联激酶抑制剂，已用于治疗特发性肺纤维化，最近根据安慰剂对照随机对照试验的结果在美国和日本获得上市许可。在这项临床试验中，与安慰剂相比，尼达尼布延缓了 SSc-ILD 的进展。

覆盖区域

本综述涵盖了 SSc-ILD 的当前药物疗法、尼达尼布的药物概况，以及尼达尼布在随机对照试验中观察到的特发性肺纤维化和 SSc-ILD 患者的疗效和安全性概况。

专家意见

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，在临床实践中适当使用抗纤维化药物仍然具有挑战性，即在哪些情况下使用它们、使用时间、如何正确使用它们以及是否与免疫抑制剂联合使用。