Systemic juvenile idiopathic arthritis (JIA) is a subtype of childhood arthritis associated with significant systemic inflammation as well as arthritis. Hyaluronic acid as a constituent of connective tissue has previously been described as a marker for arthritis in JIA in an Asian population. Mutations in hyaluronic acid synthase 2 leading to accumulation of hyaluronic acid have been found to be associated with a periodic fever syndrome in Shar-Pei dogs. Hyaluronic acid has therefore been suspected as a risk factor for systemic inflammation in humans.