Systemic sclerosis & Raynaud's
Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort
Authors' conclusion
As about 40% of untreated patients show ILD progression after 3 years and effective and safe therapies for SSc-ILD are available, our results support a change in clinical practice in selecting patients for treatment.
Study context
- Population
- Patients with SSc-ILD in two well characterised SSc-ILD cohorts234 untreated in progression analysis
- Participants
- 386 patients
- Exposure
- Untreated vs Treated
- Untreatedn = 287
- Outcome
- ILD progression in untreated patients (forced vital capacity (FVC) decline ≥10%, or FVC decline 5%-9% with DLCO decline ≥15% over 12±3 months, or start of any ILD-modifying treatment, or increase in ILD extent)
- Follow-up
- 3 years
Methodological detailstwo-centre, real-life cohort, multivariable logistic regression, multivariate Cox regression
- Two-centre
- Real-life cohort
- Multivariable logistic regression
- Multivariate Cox regression
Main finding
As reported in the abstract
3-year cumulative incidence of ILD progression in untreated patients
Untreated
39.9%
cumulative incidence · CI 32.9–46.2
Observational data: associations, not effects.
Further findings
- Untreated at baseline
- 74% · 287
Factors independently associated with no interstitial lung disease (ILD)-modifying treatment at baseline
- Anticentromere antibodies, OR (p=0.001)
- 6.75 (2.16-21.14)
- Limited extent of ILD, OR (p=0.015)
- 2.39 (1.19-4.82)
- Longer disease duration, OR (p=0.038)
- 1.04 (1.00-1.08)
- Higher DLCO, OR (p=0.005)
- 1.02 (1.01-1.04)
- Multivariable logistic regression
Original publication
Characteristics and disease course of untreated patients with interstitial lung disease associated with systemic sclerosis in a real-life two-centre cohort. RMD Open. Published 9 January 2024. doi:10.
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