George Peter M, Rennison-Jones Christian, Benvenuti Giacomo, Sifostratoudaki Aliki, Ottink Finja A, Bou-Zeid Wassim, Ledda Roberta E, Abul Kadir Roqia F, Johari Bushra, Fernandez Claire, Harston George W J, Joly Olivier, Gerry Stephen, Devaraj Anand
Department of Interstitial Lung Disease, Royal Brompton Hospital, London, UK.
National Heart and Lung Institute, Imperial College, London, UK.
ERJ Open Res. 2024 Dec 2;10(6). doi: 10.1183/23120541.00570-2024. eCollection 2024 Nov.
In patients with idiopathic pulmonary fibrosis (IPF) there is a need to identify biomarkers that 1) are associated with increased risk of adverse outcome and 2) can be used to monitor treatment response or identify disease progression over time.
Two consecutive cohorts of patients with IPF were accessed from the Open Source Imaging Consortium database. Automated computed tomography (CT) biomarkers of disease severity incorporating fibrotic and pulmonary vascular features (the reticulovascular score and weighted reticulovascular score (WRVS)) were studied. Relationships between imaging biomarkers, lung function and survival were analysed.
In separate test and validation cohorts, 168 and 176 patients with IPF respectively (median survival 2.6 years) were studied. A threshold of WRVS ≥15% at baseline CT was most strongly associated with transplant-free survival (HR 3.00, 95% CI 1.47-6.10, p=0.002) when adjusted for baseline forced vital capacity (FVC) and age. In patients with 12-month follow-up CT and lung function tests (n=89) an increase in 3% of WRVS (the minimal clinically important difference) was also significantly associated with reduced survival independent of FVC, and outperformed visual evaluation of progressive fibrosis.
WRVS is an automated CT biomarker which can identify patients with IPF at increased risk of progression and is able to reliably capture disease progression over time.
在特发性肺纤维化（IPF）患者中，需要确定生物标志物，这些标志物应具备以下两点：1）与不良结局风险增加相关；2）可用于监测治疗反应或识别随时间推移的疾病进展。
从开源影像联盟数据库中获取了两个连续的IPF患者队列。研究了包含纤维化和肺血管特征的疾病严重程度的自动计算机断层扫描（CT）生物标志物（网状血管评分和加权网状血管评分（WRVS））。分析了影像生物标志物、肺功能和生存率之间的关系。
在单独的测试队列和验证队列中，分别对168例和176例IPF患者（中位生存期2.6年）进行了研究。在根据基线用力肺活量（FVC）和年龄进行调整后，基线CT时WRVS≥15%的阈值与无移植生存期的相关性最强（风险比3.00，95%可信区间1.47 - 6.10，p = 0.002）。在接受12个月随访CT和肺功能测试的患者（n = 89）中，WRVS增加3%（最小临床重要差异）也与不依赖FVC的生存率降低显著相关，并且在评估进行性纤维化方面优于视觉评估。
WRVS是一种自动CT生物标志物，它可以识别IPF患者中进展风险增加的患者，并能够可靠地捕捉随时间推移的疾病进展。
