Biological treatment in adult-onset Still's disease

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder that is characterised by high spiking fever, arthritis or arthralgia, and evanescent rash. Many other systemic manifestations may occur. Pathogenesis of AOSD remains partially unknown but a major role has been recently attributed to pro-inflammatory Th1 cytokines, including tumour necrosis factor-α (TNF-α), interleukin (IL)-1, IL-6 and IL-18. Despite limited evidence, mainly based on observational studies and the extrapolation to AOSD of the results of a few controlled studies that have been conducted in children with systemic juvenile idiopathic arthritis, biological agents represent a major therapeutic advances for patients with AOSD refractory to conventional treatment or presenting life-threatening manifestations. Both IL-1 and IL-6 blockade may be more effective than TNF-α blockers. Although debatable, therapeutic strategies are proposed.