Systemic sclerosis & Raynaud's
Serum type I interferon score as a disease activity biomarker in patients with diffuse cutaneous systemic sclerosis: a retrospective cohort study
Authors' conclusion
Serum IFN score assessment for patients with diffuse cutaneous systemic sclerosis could identify patients with high disease activity who are more likely to have worse 12-month prognosis and overall survival.
Study context
- Population
- Adults with dcSSc in the US Prospective Registry of Early Systemic Sclerosis (PRESS) registry (incident cohort) or the UK STRIKE registry (prevalent cohort), and healthy controls110 incident, 72 prevalent
- Exposure
- Interferon (IFN)-high vs IFN-low serum IFN score
- Healthy controlsn = 32
- Outcome
- Main outcome measures: baseline and MCIDs for modified Rodnan Skin Score, FVC, DLCO and HAQ-DI
- Follow-up
- 12 months
Methodological detailstwo cohorts (incident and prevalent)
- Two cohorts (incident and prevalent)
Main finding
As reported in the abstract
Classified IFN-high
Observational data: associations, not effects.
Further findings
| Outcome | IFN-high | IFN-low | P value |
|---|---|---|---|
| Baseline mean predicted FVC (incident cohort)IFN-high vs IFN-low | 72.0% (SD 18.9) | 85.3% (SD 18.5) | P = 0.0028 |
| Baseline DLCO (incident cohort)IFN-high vs IFN-low · mean | 56.8% (SD 21.6) | 76.6% (SD 25.3) | P = 0.0008 |
| Baseline HAQ-DI (incident cohort)IFN-high vs IFN-low · median | 1.4 (IQR 0.8–2.0) | 0.8 (IQR 0.4–1.5) | P = 0.0033 |
| Disease duration (prevalent cohort)IFN-high vs IFN-low · median, years | 2.2 (IQR 0.7–8.2) | 5.0 (IQR 1.9–10.0) | P = 0.035 |
| 5% relative worsening of FVC (prevalent cohort) · 12 monthsIFN-high vs IFN-low · independent of baseline FVC or immunosuppression | 39% | 17% | P = 0.051 |
| Cumulative 5-year mortality · 5 yearsIFN-high vs IFN-low | 24.9% (95% CI 14.9–39.7) | 8.6% (95% CI 3.6–19.9) | P = 0.052 |
- Baseline mean predicted forced vital capacity (FVC) (incident cohort): differences persisted at last follow-up (median 34 months)
- Baseline DLCO (incident cohort): differences persisted at last follow-up (median 34 months)
- 5% relative worsening of FVC (prevalent cohort): 23 IFN-high vs 41 IFN-low patients
Original publication
Serum type I interferon score as a disease activity biomarker in patients with diffuse cutaneous systemic sclerosis: a retrospective cohort study. Lancet Rheumatol. Published 31 March 2025. doi:10.
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