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Radiology, Mar 2025, 314 (3) | https://doi.org/10.1148/radiol.242292
Evaluation of Autoimmune Features in Patients with Idiopathic Pulmonary Fibrosis and Pathologic Usual Interstitial Pneumonia: Implications for CT Patterns and Prognosis
Sohee Park1, Jooae Choe1, Ho Cheol Kim2, Hye Jeon Hwang1, Eun Jin Chae1, Joon Beom Seo1
1 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
2 Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
Address correspondence to: J.C.
1 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
2 Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
Address correspondence to: J.C.
Abstract
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]; P = .005) and serologic (20% [17 of 87] vs 9.8% [12 of 123]; P = .04) and IPAF (4.1% [five of 123] vs 21% [18 of 87]; P < .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; P = .58). Lymphoid follicles (HR, 0.59; 95% CI: 0.37, 0.93; P = .02), CT signs for CTD (HR, 0.31; 95% CI: 0.09, 0.99; P = .047), and use of an antifibrotic agent (HR, 0.31; 95% CI: 0.19, 0.51; P < .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; P < .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.
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]; P = .005) and serologic (20% [17 of 87] vs 9.8% [12 of 123]; P = .04) and IPAF (4.1% [five of 123] vs 21% [18 of 87]; P < .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; P = .58). Lymphoid follicles (HR, 0.59; 95% CI: 0.37, 0.93; P = .02), CT signs for CTD (HR, 0.31; 95% CI: 0.09, 0.99; P = .047), and use of an antifibrotic agent (HR, 0.31; 95% CI: 0.19, 0.51; P < .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; P < .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.
논문정보
- Research article
- 2025년 03월 (BRIC 등록일 2025-05-09)
- 국내 연구진
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