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Abstract Number: 2034

Resolving Phenotypic and Prognostic Differences in Interstitial Lung Disease Related to Systemic Sclerosis by Computed Tomography-based Radiomics

Janine Schniering1, Malgorzata Maciukiewicz1, Hubert Gabrys2, Matthias Brunner1, Christian Blütghen3, Chantal Meier1, Sophie Braga-Lagache4, Anne-Christine Ulgry4, Manfred Heller4, Oliver Distler1, Matthias Guckenberger2, Havard Fretheim5, Anna-Maria Hoffmann-Vold5, Christos Nakas6, Thomas Frauenfelder3, Stephanie Tanadini-Lang2 and Britta Maurer7, 1Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Switzerland, Zurich, Switzerland, 2Department of Radiation Oncology, University Hospital Zurich, Switzerland, Zurich, Switzerland, 3Institute of Diagnostic and Interventional Radiology, University Hospital Zurich, Switzerland, Zurich, Switzerland, 4Proteomics and Mass Spectrometry Core Facility, Department for BioMedical Research (DBMR), University of Bern, Switzerland, Bern, Switzerland, 5Department of Rheumatology, Oslo University Hospital, and Institute of Clinical Medicine, University of Oslo, Norway, Oslo, Norway, 6Laboratory of Biometry, University of Thessaly, Volos, Greece, Volos, Greece, 7Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, Switzerland, Zurich

Meeting: ACR Convergence 2020

Keywords: Biomarkers, Computed tomography (CT), Imaging, interstitial lung disease, Scleroderma, Systemic

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Session Information

Date: Monday, November 9, 2020

Title: Systemic Sclerosis & Related Disorders – Clinical I: Stratification Strategies for Clinical Trials (2033–2037)

Session Type: Abstract Session

Session Time: 3:00PM-3:50PM

Background/Purpose: Radiomics describes the in-depth analysis of tissue phenotypes by computational retrieval of high-dimensional quantitative imaging features including tissue intensity, texture, and wavelet characteristics. Here, we aimed to evaluate high-resolution computed tomography (HRCT)-based radiomics for disease phenotyping and risk stratification in interstitial lung disease related to systemic sclerosis (SSc-ILD).

Methods: In this study, we investigated two independent, prospectively followed SSc-ILD cohorts including 90 patients (76.7% female, median age 57.5 years) from the University Hospital Zurich and 66 patients (75.8% female, median age 61.0 years) from the Oslo University Hospital. For every subject, we defined and extracted 1,386 radiomic features from semi-automated segmented HRCT images including 17 intensity, 137 texture, and 1,232 wavelet features using our in-house developed radiomics software Z-Rad. After filtering of robust radiomic features, we performed unsupervised k-Means clustering and supervised prediction modelling to 1) identify homogeneous imaging-based groups without any a priori assumptions, and 2) to derive a quantitative radiomic risk score for progression-free survival (PFS) in SSc-ILD. PFS was defined as the time to a relative decline in FVC% predicted ≥ 15%. Associations with clinical characteristics at baseline and PFS among the obtained clusters and risk score groups were assessed by Fisher’s Exact and Mann-Whitney U test, or univariable Cox regression, respectively.

Results: Unsupervised cluster analysis of 1,355 robust radiomic features revealed two distinct patient clusters based on their radiomic profiles. The two patient clusters exhibited significant differences in their lung disease-specific baseline parameters, but not in serological or demographic characteristics. Cluster 2 presented a more severe ILD phenotype than cluster 1, and was significantly associated (p< 0.05) with a worse restrictive ventilation defect, pulmonary hypertension, a fibrosis extent on HRCT of ≥ 20%, and certain visual HRCT ILD patterns, including UIP radiological subtype and honeycombing. The clusters further significantly differed in their outcome with cluster 2 showing a decreased PFS and thus a higher risk of faster disease progression (p=0.001). We next derived a quantitative radiomic risk score (qRISSc) composed of the sum of 26 equally weighted radiomic features that accurately predicted PFS and showed prognostic power in both study cohorts (C-index = 0.67 for Zurich, 0.71 for Oslo). We also compared the prognostic potential of qRISSc to existing SSc-ILD stratification tools, including subgrouping of patients based on HRCT (< 20% or ≥20% fibrosis) or the FVC% predicted threshold of < 70%, respectively. In both cohorts, neither HRCT- nor FVC%-based risk stratification was prognostic for future lung function decline, overall indicating the superiority of qRISSc over current prognostic measures.

Conclusion: Our data suggests that radiomic features and radiomics-derived scores can capture important phenotypic and prognostic information thus showing great potential for risk stratification in SSc-ILD.


Disclosure: J. Schniering, None; M. Maciukiewicz, None; H. Gabrys, None; M. Brunner, None; C. Blütghen, None; C. Meier, None; S. Braga-Lagache, None; A. Ulgry, None; M. Heller, None; O. Distler, Actelion, 2, 5, 8, Bayer, 2, 5, 8, Boehringer Ingelheim, 2, 5, 8, Medscape, 5, 8, Novartis, 8, Roche, 5, 8, Menarini, 8, Mepha, 8, MSD, 5, 8, iQone, 8, Pfizer, 5, 8, AbbVie, 5, Acceleron Pharma, 5, Amgen, 5, AnaMar, 5, Arxx Therapeutics, 5, Beacon Discovery, 5, Blade Therapeutics, 5, CSL Behring, 5, ChemomAb, 5, Corpus Pharma, 5, Curzion Pharmaceuticals, 5, Ergonex Pharma, 5, Mitsubishi Tanabe Pharma, 2, 5, Kymera Therapeutics, 2, 5, Catenion, 5, Galapagos NV, 5, GlaxoSmithKline, 5, Glenmark Pharmaceuticals, 5, Inventiva, 5, Italfarmaco, 5, Lilly, 5, Sanofi, 5, UCB, 5, IQVIA, 5, Medac, 5, Target BioScience, 5, Patent issued, 9; M. Guckenberger, None; H. Fretheim, Actelion, 9, GSK, 9, Bayer, 9; A. Hoffmann-Vold, Roche, 8, Actelion, 8, Bayer, 8, Boehringer-Ingelheim, 2, 8; C. Nakas, None; T. Frauenfelder, None; S. Tanadini-Lang, None; B. Maurer, AbbVie, 2, Protagen, 2, Novartis Biomedical Research, 2, Pfizer, 9, Roche, 9, Actelion, 9.

To cite this abstract in AMA style:

Schniering J, Maciukiewicz M, Gabrys H, Brunner M, Blütghen C, Meier C, Braga-Lagache S, Ulgry A, Heller M, Distler O, Guckenberger M, Fretheim H, Hoffmann-Vold A, Nakas C, Frauenfelder T, Tanadini-Lang S, Maurer B. Resolving Phenotypic and Prognostic Differences in Interstitial Lung Disease Related to Systemic Sclerosis by Computed Tomography-based Radiomics [abstract]. Arthritis Rheumatol. 2020; 72 (suppl 10). https://acrabstracts.org/abstract/resolving-phenotypic-and-prognostic-differences-in-interstitial-lung-disease-related-to-systemic-sclerosis-by-computed-tomography-based-radiomics/. Accessed .
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