Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons - Columbia University Irving Medical Center, New York, New York, USA.
Curr Opin Rheumatol. 2019 Nov;31(6):553-560. doi: 10.1097/BOR.0000000000000660.
Systemic sclerosis (SSc) is a heterogeneous disease with a variable disease course. Interstitial lung disease (ILD) is one of the leading causes of morbidity and mortality in patients with SSc. The present review highlights recent advances in the classification, diagnosis, and early detection of SSc-associated ILD (SSc-ILD).
Risk stratification through measurement of disease extent on high-resolution computed tomography (HRCT) of the chest, longitudinal declines in pulmonary function tests (PFTs), and mortality prediction models have formed the basis for classifying clinically significant ILD. HRCT may be preferred over PFTs for screening, as PFTs lack sensitivity and have a high false-negative rate. Novel imaging modalities and biomarkers hold promise as adjunct methods for assessing the presence and severity of SSc-ILD, and predicting risk for progressive disease. Further validation is required prior to their use in clinical settings.
Classification of SSc-ILD has shifted to a personalized approach that considers an individual patient's probability of progressive disease through identification of risk factors, measurement of disease extent on HRCT, longitudinal declines in PFTs, and mortality prediction models. There remains an unmet need to develop screening guidelines for SSc-ILD.
系统性硬化症（SSc）是一种异质性疾病，其病程多变。间质性肺疾病（ILD）是 SSc 患者发病率和死亡率的主要原因之一。本综述重点介绍了 SSc 相关间质性肺疾病（SSc-ILD）的分类、诊断和早期检测方面的最新进展。
通过胸部高分辨率计算机断层扫描（HRCT）测量疾病程度、肺功能测试（PFT）的纵向下降以及死亡率预测模型进行风险分层，为临床显著ILD 的分类奠定了基础。HRCT 可能优于 PFT 用于筛查，因为 PFT 缺乏敏感性且假阴性率高。新型成像方式和生物标志物有望成为评估 SSc-ILD 存在和严重程度以及预测疾病进展风险的辅助方法。在临床应用之前，还需要进一步验证。
SSc-ILD 的分类已转向个性化方法，通过识别风险因素、HRCT 测量疾病程度、PFT 的纵向下降以及死亡率预测模型，考虑个体患者进展性疾病的概率。仍然需要制定 SSc-ILD 的筛查指南。
