Division of Rheumatology, Mayo Clinic, Rochester, MN, USA.
Division of Rheumatology, Mayo Clinic, Scottsdale, AZ, USA.
Expert Rev Clin Immunol. 2023 Jun;19(6):613-626. doi: 10.1080/1744666X.2023.2198212. Epub 2023 Apr 10.
Interstitial lung disease (ILD) is the leading cause of mortality in patients with systemic sclerosis (SSc). Risk of developing progressive ILD is highest among patients with diffuse cutaneous disease, positive anti-topoisomerase I antibody, and elevated acute phase reactants. With the FDA approval of two medications and a pipeline of novel therapeutics in trials, early recognition and intervention is critical. High-resolution computed tomography of the chest is the current gold standard test for diagnosis of ILD. Yet, it is not offered as a screening tool to all patients due to which ILD can be missed in up to a third of patients. There is a need to develop and validate more innovative screening modalities.
In this review, we provide an overview of screening and diagnosis of SSc-ILD, highlighting the recent innovations particularly the role of soluble serologic, radiomic (quantitative lung imaging, lung ultrasound), and breathomic (exhaled breath analysis) biomarkers in the early detection of SSc-ILD.
There is remarkable progress in the development of new radiomics and serum biomarkers in diagnosing SSc-ILD. There is an urgent need for conceptualizing and testing composite ILD screening strategies that incorporate these biomarkers.
间质性肺病（ILD）是系统性硬化症（SSc）患者死亡的主要原因。弥漫性皮肤疾病、抗拓扑异构酶 I 抗体阳性和急性期反应物升高的患者发生进行性 ILD 的风险最高。随着两种药物获得美国食品和药物管理局（FDA）批准，以及一系列新型治疗药物正在临床试验中，早期识别和干预至关重要。胸部高分辨率计算机断层扫描是目前诊断 ILD 的金标准检测方法。然而，由于并非所有患者都接受了该检测，因此多达三分之一的患者可能会漏诊 ILD。因此，需要开发和验证更具创新性的筛查方法。
在这篇综述中，我们概述了 SSc-ILD 的筛查和诊断，重点介绍了可溶性血清学、放射组学（定量肺部成像、肺部超声）和呼吸组学（呼气分析）生物标志物在 SSc-ILD 早期检测中的最新创新。
在开发用于诊断 SSc-ILD 的新型放射组学和血清学生物标志物方面取得了显著进展。迫切需要构思和测试包含这些生物标志物的综合ILD 筛查策略。
