Novel Imaging Metric Tracks Calcinosis Cutis Changes in SSc
Publication Title: Scleroderma calcinosis cutis score (SC2S): an imaging metric to quantify SSc-calcinosis cutis
Summary
- Question
- This study aimed to develop and test a novel imaging metric called the scleroderma calcinosis cutis score (SC2S) to quantify calcinosis cutis (CC) in patients with systemic sclerosis (SSc). The researchers evaluated SC2S's reproducibility and sensitivity to changes in CC volume during treatment with sodium thiosulfate (STS).
- Why it Matters
- Calcinosis cutis, a painful condition causing calcium deposits in the skin, significantly impacts the quality of life for patients with systemic sclerosis. Despite its debilitating effects, effective treatments and reliable methods to measure CC have been limited. SC2S offers a promising tool for quantifying changes in CC volume, potentially enabling more accurate tracking of treatment outcomes and advancing clinical trials for new therapies.
- Methods
- Five female patients with systemic sclerosis and calcinosis cutis were recruited for this pilot study. Participants received STS treatment, either as monthly intradermal injections or twice-daily topical cream, over 6 to 12 months. Pre- and post-treatment CT scans of the affected areas were analyzed using BioImageSuite (BIS Web), a software that uses computer-assisted image mapping to measure CC volume. The SC2S metric was compared to an existing radiologic scoring system for hand calcinosis.
- Key Findings
- SC2S demonstrated high reproducibility, with an intraclass correlation coefficient of 0.93 among assessors. CC volume changes varied across treatment sites: for example, one patient’s left buttock lesion decreased from 40 cm³ to 10 cm³ (a 75% reduction), while another patient’s right hand lesion increased from 15 cm³ to 19 cm³ (a 27% increase). Additionally, repeated measurements of CC volume showed minimal variation (≤3%) over a one-week interval. SC2S captured differences in treatment responses and highlighted its sensitivity to both volume reductions and increases.
- Implications
- SC2S could serve as a reliable tool for quantifying CC changes in systemic sclerosis patients, offering greater precision than existing methods like hand radiographs. This tool may improve clinical assessments, facilitate the evaluation of new treatments, and support the development of more targeted therapeutic approaches.
- Next Steps
- Future research should focus on automating the SC2S mapping process to reduce time-intensive manual mapping, particularly for complex areas such as hands and knees. Larger studies are needed to validate SC2S’s performance and explore its application in randomized controlled trials for systemic sclerosis-related calcinosis cutis.
- Funding Information
- This research was supported by the National Institutes of Health (awards R01 AR073270, R01 AR085316, K08 AR077689, and R03 AR083454). Additional funding was provided by Kadmon Pharmaceuticals, Ventus Pharmaceuticals, SenTry, Inc., and Plythera, Inc. Yale University also provided funding and support for this research.
Full Citation
Odell I, Cheung C, Wu M, Perez S, Dixit A, van Horn C, Hamdan M, Gunes I, Kujawski S, Lee H, Wang A, Esserman D, Zamani M, Wilson F, Onofrey J, Papademetris X, Hinchcliff M. Scleroderma calcinosis cutis score (SC2S): an imaging metric to quantify SSc-calcinosis cutis. Rheumatology 2026, 65: keag302. PMID: 42281277, PMCID: PMC13290485, DOI: 10.1093/rheumatology/keag302.
Authors
Ian Odell, MD, PhD, FAAD
First AuthorAssociate Professor of Dermatology
Monique Evangeline Hinchcliff, MD, MS
Last AuthorAssociate Professor of Medicine (Rheumatology, Allergy & Immunology)