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

Abstract

To examine whether early therapeutic intervention, compared with delayed intervention, is beneficial for patients with early SSc. 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). 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). A therapeutic 'window of opportunity' might exist in SSc patients.