Outcomes in patients with systemic sclerosis undergoing early vs delayed intervention with potential disease-modifying therapies

Objective To examine whether early therapeutic intervention, compared with delayed intervention, is beneficial for patients with early SSc. Methods This is a single-centre, retrospective cohort study of SSc patients who received CYC, MMF, MTX or tocilizumab for diffuse cutaneous SSc (dcSSc) or interstitial lung disease (ILD) within 6 years after disease onset. The patients were divided into early and delayed intervention groups based on the disease duration of <= 18 and >18 months at treatment introduction, respectively. Clinical worsening was defined as the development of any original or revised ACR Composite Response Index in SSc (CRISS) step 1 event or progressive fibrosing ILD (PF-ILD). Results There was no difference in baseline characteristics between the early (n = 25) and delayed (n = 21) intervention groups except forced vital capacity, which was better in the early vs delayed intervention groups. The early intervention group less frequently had stable pulmonary function over 1 year than did the late intervention group (odds ratio 0.087, 95% CI: 0.0079, 0.51; P = 0.003). The active disease was significantly decreased from 79% to 42% in the early intervention group (P = 0.007), but the change in the delayed intervention group was not statistically significant (68% to 42%; P = 0.11). Cumulative rates free from clinical worsening events defined by revised ACR-CRISS and PF-ILD were significantly higher in the early vs delayed intervention groups (P = 0.03 and 0.003, respectively). Conclusion A therapeutic 'window of opportunity' might exist in SSc patients.

Automated summary

This study aimed to investigate the benefits of early therapeutic intervention in patients with early SSc. The results showed that the early intervention group had a lower proportion of stable pulmonary function, a higher reduction in active disease, and significantly lower rates of clinical worsening compared to the delayed intervention group.