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Updated: Sep 18, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Radiological Insights into UIP Pattern: A Comparison Between IPF and Non-IPF Patients
Stefano Palmucci1,2, Miriam Adorna3, Angelica Rapisarda4
1Department of Medical Surgical Sciences and Advanced Technologies "GF Ingrassia", University of Catania, 95123 Catania, Italy.
Abstract:
Background/Objectives: This study aims to distinguish radiological differences between primary idiopathic Usual Interstitial Pneumonia (UIP) and secondary UIP patterns Methods: This retrospective study included patients with HRCT findings consistent with a UIP pattern. Final diagnoses were established via multidisciplinary discussion and classified as primary UIP/IPF or secondary UIP, following the 2022 ATS/ERS/JRS/ALAT guidelines. An expert thoracic radiologist (>10 years of experience), blinded to clinical data, reviewed the earliest available HRCT assessing key imaging features: honeycombing (micro-, macro- or exuberant), fibrosis distribution (symmetry, anterior-upper lobe sign, etc.), ground-glass opacities (GGO), dilatation of esophagus. Additionally, AI software AVIEW Build 1.1.46.28-win Coreline (©Coreline Soft Co., Ltd. All Rights Reserved). performed lung texture analysis, quantifying total lung volume and radiological patterns. Statistical analysis was performed to reveal results. Results: Among 53 cases, 31 were classified as IPF and 22 as secondary UIP cases. The expert radiologist achieved a diagnostic sensitivity of 82.9%, specificity of 889%, with a positive predictive value of 93.5%-in distinguishing between primary and secondary UIP. Primary UIP cases exhibited typical hallmark radiological features, including uniform honeycombing with cranio-caudal distribution (90.3%). Reticulations contributed significantly to the fibrotic texture, maintaining a consistent cranio-caudal gradient and axial symmetry (84.8%). Secondary UIP displayed more significant radiological heterogeneity, including patchy fibrosis with irregular GGO distribution (84.5% versus 53.33%); other findings-such as exuberant honeycombing, four corner sign and wedge-shaped fibrosis-were mainly observed in secondary pattern with respective percentages of 31.8%, 9% and 49%. Conclusions: Experienced thoracic radiologists, leveraging hallmark imaging features, play a critical role in improving diagnostic accuracy between primary and secondary UIP patterns.
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