Marinescu Daniel-Costin, Hague Cameron J, Muller Nestor L, Murphy Darra, Churg Andrew, Wright Joanne L, Al-Arnawoot Amna, Bilawich Ana-Maria, Bourgouin Patrick, Cox Gerard, Durand Celine, Elliot Tracy, Ellis Jennifer, Fisher Jolene H, Fladeland Derek, Grant-Orser Amanda, Goobie Gillian C, Guenther Zachary, Haider Ehsan, Hambly Nathan, Huynh James, Johannson Kerri A, Karjala Geoffrey, Khalil Nasreen, Kolb Martin, Lapointe-Shaw Lauren, Leipsic Jonathon, Lok Stacey, MacIsaac Sarah, McInnis Micheal, Manganas Helene, Marcoux Veronica, Mayo John, Morisset Julie, Scallan Ciaran, Sedlic Tony, Shapera Shane, Sun Kelly, Tan Victoria, Wong Alyson W, Zheng Boyang, Ryerson Christopher J
From the Department of Medicine, University of British Columbia, Vancouver, Canada (D.C.M., G.C.G., N.K., A.W.W., B.Z., C.J.R.); Centre for Lung Health, Vancouver General Hospital, 2775 Laurel St, 7th Fl, Vancouver, BC, Canada V5Z 1M9 (D.C.M., N.K.); Department of Radiology, University of British Columbia, Vancouver, Canada (C.J.H., N.L.M., A.M.B., J.E., J.L., J. Mayo, T.S.); Department of Radiology, St James' Hospital, Dublin, Ireland (D.M.); Department of Pathology, University of British Columbia, Vancouver, Canada (A.C., J.L.W.); Department of Radiology, McMaster University, Hamilton, Canada (A.A.A., E.H., V.T.); Department of Radiology, University of Montreal, Montreal, Canada (P.B.); Department of Medicine, McMaster University, Hamilton, Canada (G.C., N.H., M.K., S.M., C.S.); Department of Medicine, University of Toronto, Toronto, Canada (J.H.F., L.L.S., S.S., K.S.); Department of Medicine, Centre de recherche du Centre hospitalier de l'Université de Montréal, Montreal, Canada (C.D., H.M., J. Morisset); Department of Radiology, University of Calgary, Calgary, Canada (T.E., Z.G., J.H.); Department of Medical Imaging, University of Saskatchewan, Saskatoon, Canada (D.F., G.K.); Department of Medicine, University of Calgary, Calgary, Canada (A.G.O., K.A.J.); Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, University of Pittsburgh, Pittsburgh, Pa (G.C.G.); Centre for Heart Lung Innovation, St Paul's Hospital, Vancouver, Canada (D.C.M., G.C.G., A.W.W., B.Z., C.J.R.); Department of Medicine, University of Saskatchewan, Saskatoon, Canada (S.L., V.M.); and Department of Medical Imaging, University of Toronto, Toronto, Canada (M.M.).
Radiology. 2025 Feb;314(2):e241001. doi: 10.1148/radiol.241001.
Background Prognostic value of radiologic features in interstitial lung disease (ILD) has been predominantly studied in idiopathic pulmonary fibrosis, but findings vary. The relative importance of features versus guideline-defined patterns in predicting outcomes is unknown. Purpose To identify radiologic features that are independently associated with transplant-free survival beyond clinical predictive factors across all ILD subtypes, and to identify whether individual features versus patterns are more important for prognostication. Materials and Methods This is a secondary analysis of the prospective Canadian Registry for Pulmonary Fibrosis. Consecutive patients with ILD were evaluated in standardized multidisciplinary discussions between January 2021 and March 2022. Radiologic features on thin-section CT images were quantified, and guideline-defined usual interstitial pneumonia (UIP) and fibrotic hypersensitivity pneumonitis (fHP) patterns were assigned. Multivariable Cox analysis was used to assess the associations of radiologic features with transplant-free survival, and nested models were used to test the relative importance of features compared with patterns. Results A total of 1593 patients (mean age, 66 years ± 12 [SD]; 800 male) were included. The following four features were associated with transplant-free survival: extent of honeycombing (hazard ratio, 1.20; 95% CI; 1.06, 1.36 per 10% increase in lung involvement; = .005), extent of traction bronchiectasis (hazard ratio, 1.18; 95% CI: 1.10, 1.26 per 10% increase; < .001), pulmonary artery diameter (hazard ratio, 1.03; 95% CI: 1.01; 1.04 per 1-mm increase; = .002), and presence of subpleural sparing (hazard ratio, 0.76; 95% CI: 0.56, 0.96; = .03). Guideline-defined patterns were not independently associated with survival in a model that included these four radiologic features, each of which retained its prognostic value. Conclusion The extent of fibrosis was predictive of worse outcomes across all ILD subtypes in a dose-dependent fashion and independent of well-recognized clinical prognostic factors. Guideline-defined UIP and fHP patterns each helped risk-stratify patients in isolation but lost prognostic value when accounting for the extent of fibrosis, suggesting that their previous association with mortality is based on these patterns acting as surrogates for a greater extent of fibrosis. © RSNA, 2025 See also the editorial by Wells in this issue.
间质性肺疾病（ILD）影像学特征的预后价值主要在特发性肺纤维化中进行了研究，但结果存在差异。在预测结局方面，特征与指南定义的模式的相对重要性尚不清楚。目的：确定在所有ILD亚型中，除临床预测因素外，与无移植生存独立相关的影像学特征，并确定个体特征与模式对预后判断哪个更重要。材料与方法：这是对前瞻性加拿大肺纤维化登记处的二次分析。2021年1月至2022年3月期间，在标准化的多学科讨论中对连续的ILD患者进行了评估。对薄层CT图像上的影像学特征进行量化，并确定指南定义的普通型间质性肺炎（UIP）和纤维化性过敏性肺炎（fHP）模式。采用多变量Cox分析评估影像学特征与无移植生存的相关性，并使用嵌套模型测试特征与模式相比的相对重要性。结果：共纳入1593例患者（平均年龄66岁±12[标准差]；800例男性）。以下四个特征与无移植生存相关：蜂窝状改变范围（风险比，1.20；95%可信区间：肺受累每增加10%为1.06，1.36；P = 0.005）、牵拉性支气管扩张范围（风险比，1.18；95%可信区间：每增加10%为1.10，1.26；P < 0.001）、肺动脉直径（风险比，1.03；95%可信区间：每增加1mm为1.01，1.04；P = 0.002）以及胸膜下 sparing的存在（风险比，0.76；95%可信区间：0.56，0.96；P = 0.03）。在包含这四个影像学特征的模型中，指南定义的模式与生存无独立相关性，每个特征均保留其预后价值。结论：纤维化程度以剂量依赖方式预测所有ILD亚型的较差结局，且独立于公认的临床预后因素。指南定义的UIP和fHP模式各自单独有助于对患者进行风险分层，但在考虑纤维化程度时失去预后价值，这表明它们先前与死亡率的关联是基于这些模式作为更大程度纤维化的替代指标。©RSNA，2025 另见本期Wells的社论。
