A screening tool to detect interstitial lung disease in systemic sclerosis: the ILD-RISC score
Bruni C, Tofani L, Fretheim H, Liem SI, Velauthapillai A, Bjørkekjær H, Barua I, Galetti I, Garaiman A, Becker MO, Hoffmann-Vold AM, De Vries-Bouwstra J, Vonk MC, Distler JH, Matucci-Cerinic M, Distler O (2025)
Publication Type: Journal article
Publication year: 2025
Journal
Book Volume: 64
Pages Range: 6285-6293
Journal Issue: 12
DOI: 10.1093/rheumatology/keaf445
Abstract
Objectives: As a screening tool for the presence of systemic sclerosis-associated interstitial lung disease (SSc-ILD) on high-resolution computed tomography (HRCT) is missing, we aimed to develop the ILD-RISC score, a risk algorithm to guide physicians in ordering HRCTs, with specific focus on follow-up visits. Methods: The nominal group technique was used to select items for the multivariable logistic regression with backward selection. The ILD-RISC score was developed from baseline visits of the derivation cohort. After identifying a cut-off favoring sensitivity>85% from the ROC curve, it was validated in a separate cross-sectional cohort, and then applied longitudinally in a specific SSc cohort with negative baseline HRCT. Results: In the derivation cohort (533 patients), 13 variables associated with the presence of SSc-ILD on HRCT were tested. The ILD-RISC score, including FVC%, DLCO/SB%, digital ulcers ever, age and SSc autoantibodies, showed an area under the curve of 79.1% (75.3–83.0%) for the presence of SSc-ILD on HRCT. An ILD-RISC score ≥ 0.3 had sensitivity 85.6% and specificity 53.6%, as confirmed in the validation cohort (247 patients). Among 819 patients with negative baseline HRCT, 170 developed SSc-ILD: a low ILD-RISC score was detected in almost 50% of the follow-up visits, supporting the sparing of HRCTs. Conclusion: The ILD-RISC score was developed and validated to predict the presence of SSc-ILD. Thus, the ILD-RISC score may help to decide when to order HRCTs at follow-up, allowing the reduction of costs and radiation exposure, but also at the time of SSc diagnosis when resources are limited.
Involved external institutions
Radboud University Nijmegen Medical Centre / Radboudumc of voluit Radboud Universitair Medisch Centrum (UMC)
Netherlands (NL)
Leiden University Medical Center
Netherlands (NL)
Universitätsklinikum Düsseldorf
Germany (DE)
Università degli Studi di Firenze / University of Florence
Italy (IT)
Universitätsspital Zürich (USZ)
Switzerland (CH)
Hospital of Southern Norway / Sørlandet sykehus Kristiansand
Norway (NO)
Ospedale San Raffaele (früher: Centro San Raffaele del Monte Tabor Foundation)
Italy (IT)
Oslo University Hospital / Oslo Universitetssykehus Rikshospitalet
Norway (NO)
Netherlands (NL)
Leiden University Medical Center
Netherlands (NL)
Universitätsklinikum Düsseldorf
Germany (DE)
Università degli Studi di Firenze / University of Florence
Italy (IT)
Universitätsspital Zürich (USZ)
Switzerland (CH)
Hospital of Southern Norway / Sørlandet sykehus Kristiansand
Norway (NO)
Ospedale San Raffaele (früher: Centro San Raffaele del Monte Tabor Foundation)
Italy (IT)
Oslo University Hospital / Oslo Universitetssykehus Rikshospitalet
Norway (NO)
How to cite
APA:
Bruni, C., Tofani, L., Fretheim, H., Liem, S.I., Velauthapillai, A., Bjørkekjær, H.,... Distler, O. (2025). A screening tool to detect interstitial lung disease in systemic sclerosis: the ILD-RISC score. Rheumatology, 64(12), 6285-6293. https://doi.org/10.1093/rheumatology/keaf445
MLA:
Bruni, Cosimo, et al. "A screening tool to detect interstitial lung disease in systemic sclerosis: the ILD-RISC score." Rheumatology 64.12 (2025): 6285-6293.
BibTeX: Download