Systemic sclerosis & Raynaud's
Prediction of progressive fibrosing interstitial lung disease in patients with systemic sclerosis: insight from the CRDC cohort study
Authors' conclusion
This study developed the first prediction model for PF-ILD in patients with SSc-ILD, and internal validation showed favourable accuracy and stability of the model.
Study context
- Population
- SSc-ILD patients with no less than three pulmonary function tests within 6–24 months (CRDC cohort); 92.1% women, mean baseline age 46.7 years
- Participants
- 304 patients
- Comparison
- SSc-ILD patients with progressive fibrosing interstitial lung disease (PF-ILD) vs without PF-ILD
- PF-ILDn = 154
- Without PF-ILDn = 150reference
- Outcome
- Progressive fibrosing ILD (PF-ILD) prediction model
Methodological detailscohort, LASSO regression, multivariable Cox regression, internal validation with bootstrap resampling
- Cohort
- LASSO regression
- Multivariable Cox regression
- Internal validation with bootstrap resampling
Performance
As reported in the abstract
Predictors of PF-ILD in the 9-variable prediction model (multivariable Cox regression)
- Age≥50 years
- HR 1.8221; P = 0.001
- Hyperlipidemia
- HR 4.0516; P < 0.001
- Smoking history
- HR 3.8130; P < 0.001
- Diffused cutaneous systemic sclerosis (SSc) subtype
- HR 1.9753; P < 0.001
- Arthritis
- HR 2.0008; P < 0.001
- Shortness of breath
- HR 2.0487; P = 0.012
- Decreased serum immunoglobulin A level
- HR 2.3900; P = 0.002
- Positive anti-Scl-70 antibody
- HR 1.9573; P = 0.016
- Usage of cyclophosphamide/mycophenolate mofetil
- HR 0.4267; P < 0.001
- Concordance index after enhanced bootstrap resampling adjustment (internal validation)
- 0.874
- Optimism-corrected Brier Score (internal validation)
- 0.144
Further findings
Model performance, internal validation
- Concordance index after enhanced bootstrap resampling adjustment
- 0.874
- Optimism-corrected Brier Score
- 0.144
- Internal validation
Original publication
Prediction of progressive fibrosing interstitial lung disease in patients with systemic sclerosis: insight from the CRDC cohort study. RMD Open. Published 22 March 2024. doi:10.
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