Early IL-1 inhibition and long-term remission in childhood-onset Still's disease: a real-world cohort study.

Background: Childhood-onset Still's disease is a severe autoinflammatory disease characterized by systemic inflammation and risk of macrophage activation syndrome (MAS). Early cytokine-targeted therapy, particularly interleukin-1 (IL-1) inhibition, has been proposed to modify disease trajectory and improve long-term outcomes. Methods: We conducted a single-center retrospective cohort study of children with childhood-onset Still's disease followed between January 2018 and December 2025. Clinical inactive disease (CID) was defined according to Wallace criteria. Treatment patterns, biologic switching, MAS occurrence, biomarker normalization, and treatment-free remission were evaluated. Results: Eighteen patients were included (50% female), with a median age at symptom onset of 6.5 years. First-line biologic therapy consisted predominantly of IL-1 inhibition [94.4% ( Conclusion: Early IL-1-targeted therapy was associated with rapid disease control, effective corticosteroid sparing, and high rates of treatment-free remission in childhood-onset Still's disease. These findings support early cytokine inhibition as a cornerstone of treat-to-target strategies and suggest a potential disease-modifying effect in Still's disease.