Adult-onset Still's disease: Advances in the treatment

Abstract

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder mainly characterized by persistent high spiking fevers, evanescent rash, and joint involvement. The pathogenesis of AOSD is only partially known, but pro-inflammatory cytokines such as tumor necrosis factor (TNF)-α, interleukin (IL)-1, IL-6, IL-18, and IFN-γ seem to play a major role in this disorder. AOSD is at the crossroad of auto-inflammatory syndromes and autoimmune diseases. It is diagnosed by exclusion to determine the presence of high serum ferritin levels, which is usually >1000 μg/L. AOSD is generally treated with non-steroidal anti-inflammatory drugs, corticosteroids, and disease-modifying anti-rheumatic drugs (DMARDs). Although information on biologic therapy in the management of AOSD is scarce, these drugs represent a major breakthrough in the management of patients with AOSD refractory to corticosteroids or conventional DMARDs or in patients presenting life-threatening manifestations. In this regard, TNF-α, IL-1, and IL-6 antagonists had been proved effective in patients with AOSD.

Keywords: Adult-onset Still's disease; Anti-IL-6 receptor antibody-tocilizumab; Biologic agents; DMARDs; IL-1 receptor antagonist-anakinra; Macrophage activation syndrome; TNF-α inhibitors.