Park Sohee, Choe Jooae, Kim Ho Cheol, Hwang Hye Jeon, Chae Eun Jin, Seo Joon Beom
Department of Radiology and Research Institute of Radiology, University of Ulsan College of Medicine, Asan Medical Center, 86 Asanbyeongwon-Gil, Songpa-Gu, Seoul 05505, Korea.
Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea.
Radiology. 2025 Mar;314(3):e242292. doi: 10.1148/radiol.242292.
Background The clinical, radiologic, and prognostic implications of interstitial pneumonia with autoimmune features (IPAF) in patients with idiopathic interstitial pneumonia and pathologic usual interstitial pneumonia (UIP) have not been fully evaluated. Purpose To compare autoimmune features according to CT patterns for the diagnosis of idiopathic pulmonary fibrosis (IPF) and to assess the diagnostic and prognostic implications of IPAF in patients with IPF-UIP. Materials and Methods This retrospective study included patients with UIP confirmed by surgical lung biopsy between January 2013 and February 2020. Data regarding clinical, radiologic, and pathologic autoimmune features were collected, and patients were diagnosed with IPAF according to current guidelines. CT signs for connective tissue disease (CTD; anterior upper lobe, straightedge, and exuberant honeycombing signs) were also evaluated. Overall survival (OS) was evaluated using Cox proportional hazards models. Results Among 210 patients included (median age, 64 years; IQR, 60-68 years; 158 male patients), 23 (11.0%) had IPAF. Patients with an alternative diagnosis or CT pattern indeterminate for UIP showed a higher prevalence of autoimmune features that were pathologic (38% [33 of 87] vs 20.3% [25 of 123]; = .005) and serologic (20% [17 of 87] vs 9.8% [12 of 123]; = .04) and IPAF (4.1% [five of 123] vs 21% [18 of 87]; < .001) compared with patients with UIP or probable UIP pattern. However, IPAF was not predictive of OS (hazard ratio [HR], 0.81; 95% CI: 0.38, 1.72; = .58). Lymphoid follicles (HR, 0.59; 95% CI: 0.37, 0.93; = .02), CT signs for CTD (HR, 0.31; 95% CI: 0.09, 0.99; = .047), and use of an antifibrotic agent (HR, 0.31; 95% CI: 0.19, 0.51; < .001) were independently associated with higher OS, and greater extent of fibrosis on CT scans was associated with worse OS (HR, 1.08; 95% CI: 1.05, 1.11; < .001). Conclusion In patients with IPF-pathologic UIP, serologic and pathologic autoimmune features were associated with indeterminate or alternative CT patterns. Certain histopathologic and radiologic autoimmune features, but not current IPAF criteria, were associated with survival. © RSNA, 2025 See also the editorial by Ackman in this issue.
特发性间质性肺炎和病理表现为普通型间质性肺炎（UIP）患者中，具有自身免疫特征的间质性肺炎（IPAF）的临床、影像学及预后意义尚未得到充分评估。目的：根据CT表现比较特发性肺纤维化（IPF）诊断中的自身免疫特征，并评估IPAF在IPF-UIP患者中的诊断及预后意义。材料与方法：这项回顾性研究纳入了2013年1月至2020年2月间经外科肺活检确诊为UIP的患者。收集临床、影像学及病理自身免疫特征的数据，并根据现行指南对患者进行IPAF诊断。还评估了结缔组织病（CTD）的CT征象（上叶前部、直边及蜂窝状影明显）。采用Cox比例风险模型评估总生存期（OS）。结果：纳入的210例患者（中位年龄64岁；四分位间距，60-68岁；男性患者158例）中，23例（11.0%）患有IPAF。诊断存疑或CT表现不能确定为UIP的患者，其病理（38%[87例中的33例] vs 20.3%[123例中的25例]；P = .005）和血清学（20%[87例中的17例] vs 9.8%[123例中的12例]；P = .04）自身免疫特征以及IPAF（4.1%[123例中的5例] vs 21%[87例中的18例]；P < .001）的患病率均高于UIP或可能为UIP表现的患者。然而，IPAF不能预测OS（风险比[HR]，0.81；95%可信区间：0.38，1.72；P = .58）。淋巴滤泡（HR，0.59；95%可信区间：0.37，0.93；P = .02）、CTD的CT征象（HR，0.31；95%可信区间：0.09，0.99；P = .047）以及使用抗纤维化药物（HR，0.31；可信区间：0.19，0.51；P < .001）与较高的OS独立相关，CT扫描上纤维化范围越大，OS越差（HR，1.08；95%可信区间：1.05，1.11；P < .001）。结论：在IPF-病理UIP患者中，血清学和病理自身免疫特征与不确定或其他CT表现相关。某些组织病理学和影像学自身免疫特征而非现行IPAF标准与生存相关。©RSNA，2025 另见本期Ackman的社论。
