Objective:
To analyze the progression rates and severity of interstitial lung disease (ILD) in patients with late systemic sclerosis (SSc) compared to those with earlier-onset disease.
Approach:
- Study Design: A multicenter study analyzing data from 969 patients with SSc without prevalent ILD at baseline, enrolled in the Canadian Scleroderma Research Group cohort from 2004 to 2020.
- Patient Follow-Up: Patients were followed for a median of 2.4 years, with 199 (21%) developing incident ILD.
- Definitions: Late SSc was defined as disease duration ≥ 7 years from first non-Raynaud manifestation.
Key Findings:
- Incidence rate of ILD was lower in late SSc (3.7 per 100 person-years) compared to earlier SSc (5.4 per 100 person-years).
- Disease severity metrics, including FVC and DLCO values, were similar between late- and early-onset groups.
- Imaging findings showed comparable patterns of ground glass opacities and fibrotic changes between groups.
- Risk factors for developing late-onset ILD included male sex, non-White ethnicity, diffuse cutaneous involvement, and specific autoantibodies.
- No statistically significant difference in progression rates between late- and earlier-onset groups.
Interpretation:
Limitations:
- Non-systematic HRCT screening.
- Exclusion of nearly 50% of the study population from progression analyses due to missing pulmonary function test results.
- Limited data on medication dosing and duration.
- Small number of treated patients restricted analysis of treatment effectiveness.
Conclusion:
Sources:
This content is an AI-generated, fully rewritten summary based on a published scholarly article. It does not reproduce the original text and is not a substitute for the original publication. Readers are encouraged to consult the source for full context, data, and methodology.
