Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma...

Area of Science:

• Rheumatology
• Dermatology
• Clinical Trials

Background:

• Systemic sclerosis, particularly diffuse cutaneous systemic sclerosis (dcSSc), is characterized by skin thickening.
• Identifying patients with progressive skin thickening is crucial for effective management and clinical trial stratification.

Purpose of the Study:

• To identify and describe early dcSSc patients with progressive skin thickening.
• To develop prediction models for skin thickness progression over 12 months.
• To inform the design and recruitment strategies for future randomized controlled trials (RCTs) in dcSSc.

Main Methods:

• Utilized data from the European Scleroderma Observational Study, involving 326 patients.
• Defined 'progressors' as those with a 5-unit and 25% increase in modified Rodnan skin score (mRSS) over 12 months.
• Developed logistic regression models and evaluated their predictive performance using ROC curves (AUC, accuracy, PPV).

Main Results:

• 66 patients (22.5%) showed progressive skin thickening.
• Progressors had shorter disease duration and lower baseline mRSS compared to non-progressors.
• A model incorporating mRSS, skin thickening duration, and anti-RNA polymerase III (Pol3+) positivity achieved 71% accuracy and 0.711 AUC.

Conclusions:

• Two prediction models for progressive skin thickening in dcSSc were successfully derived.
• These models can be applied in clinical practice and for enriching cohorts in RCTs.
• The models will guide patient selection for upcoming dcSSc clinical trials.