The excellent Review of systemic sclerosis-associated interstitial lung disease (SSc-ILD) by Apostolos Perelas and colleagues in this issue is testament to the large body of work over several decades that has helped to better define the frequency, effects, and management of interstitial lung disease (ILD) in systemic sclerosis (SSc). Translational research has also elucidated key aspects of aetiopathogenesis. The progression of ILD in SSc is highly variable, and we are now able to identify the patients most at risk for severe ILD and determine who is more likely to have an indolent course on the basis of clinical and laboratory characteristics. Important shared mechanisms underpin the progression of ILD and its clinical effects, which also operate in idiopathic pulmonary fibrosis and other types of progressive ILD. These processes probably explain the congruent treatment benefit from nintedanib that was shown in 2019 to reduce the rate of progression of ILD in SSc and other forms of progressive lung fibrosis. These results build on the established clinical benefits of antifibrotic drugs such as nintedanib and pirfenidone in idiopathic pulmonary fibrosis.

中文翻译：

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系统性硬化相关性间质性肺疾病的循证疗法面临的挑战。

Apostolos Perelas及其同事在此问题上对系统性硬化症相关性间质性肺病（SSc-ILD）进行了出色的综述，这证明了数十年来的大量工作，有助于更好地确定间质性的频率，影响和管理全身性硬化症（SSc）中的肺部疾病（ILD）。转化研究还阐明了发病机理的关键方面。SSc中ILD的进展是高度可变的，我们现在能够根据临床和实验室特征，识别出患严重ILD风险最高的患者，并确定谁更可能接受惰性治疗。重要的共享机制是ILD进展及其临床效应的基础，这些机制也可用于特发性肺纤维化和其他类型的进行性ILD。这些过程可能解释了nintedanib在治疗中的益处，该药物在2019年被证明可以降低SSc和其他形式的进行性肺纤维化中ILD的进展速度。这些结果建立在抗纤维化药物（如nintedanib和吡非尼酮）在特发性肺纤维化中已确立的临床益处的基础上。