Columbia University Irving Medical Center, New York, New York.
University of Michigan, Ann Arbor.
Arthritis Rheumatol. 2020 Nov;72(11):1892-1896. doi: 10.1002/art.41415. Epub 2020 Sep 22.
Interstitial lung disease (ILD) is the leading cause of death in patients with systemic sclerosis (SSc). Although pulmonary function tests (PFTs) are commonly used to screen for ILD in patients with SSc, studies have shown that they lack sensitivity for the detection of ILD in general SSc cohorts. This study was undertaken to assess the performance characteristics of PFTs for the detection of ILD in patients with early diffuse cutaneous SSc (dcSSc), a population at high risk for the development of ILD.
We performed a retrospective cohort study of patients enrolled in the Prospective Registry of Early Systemic Sclerosis at 11 sites in the US between April 2012 and January 2019. Patients were included if they underwent spirometry and high-resolution computed tomography (HRCT) of the chest. We calculated the performance characteristics of PFTs for the detection of ILD on HRCT.
The study included 212 patients, 54% of whom had radiographic ILD. For the detection of ILD on HRCT imaging, a forced vital capacity (FVC) <80% predicted had a sensitivity of 63%. The combination of FVC <80% predicted or diffusing capacity for carbon monoxide (DLco) <80% predicted improved the sensitivity to 85%. An FVC <80% predicted had a negative predictive value (NPV) of 61%, while the combination of FVC <80% predicted or DLco <80% predicted had an NPV of 70%.
PFTs alone are an inadequate screening tool for the diagnosis of ILD in patients with early dcSSc. HRCT should be part of the ILD screening algorithm in patients with dcSSc.
间质性肺病（ILD）是系统性硬皮病（SSc）患者死亡的主要原因。虽然肺功能检查（PFTs）常用于筛查 SSc 患者的 ILD，但研究表明，它们对一般 SSc 队列中 ILD 的检测缺乏敏感性。本研究旨在评估 PFTs 在早期弥漫性皮肤型 SSc（dcSSc）患者中检测 ILD 的性能特征，这些患者发生 ILD 的风险较高。
我们对 2012 年 4 月至 2019 年 1 月期间在美国 11 个地点参加早期系统性硬皮病前瞻性注册研究的患者进行了回顾性队列研究。如果患者接受了肺量测定和胸部高分辨率计算机断层扫描（HRCT），则将其纳入研究。我们计算了 PFTs 在 HRCT 上检测 ILD 的性能特征。
该研究纳入了 212 例患者，其中 54%的患者存在放射性 ILD。对于 HRCT 成像上的 ILD 检测，用力肺活量（FVC）<80%预计值的敏感性为 63%。将 FVC<80%预计值或一氧化碳弥散量（DLco）<80%预计值结合起来可将敏感性提高至 85%。FVC<80%预计值的阴性预测值（NPV）为 61%，而 FVC<80%预计值或 DLco<80%预计值的组合的 NPV 为 70%。
单独使用 PFTs 是筛查早期 dcSSc 患者 ILD 的不充分工具。在 dcSSc 患者中，HRCT 应作为 ILD 筛查算法的一部分。
