Adult-onset Still’s disease (AOSD) is characterised by a group of clinical and laboratory findings indicating systemic inflammation. 1 Recent data support the hypothesis that interleukin 1 is essential in mediating inflammation, particularly in recalcitrant AOSD. 2 3 From December 2003 we treated 10 patients with AOSD diagnosed according to proposed criteria. 4 Four of these 10 consecutive patients, 4 had refractory AOSD with persistently active disease, despite administration of high-dose steroids. Herein, we report that all four patients rapidly responded to administration of anakinra (100 mg/day) and were promptly weaned from high-dose steroids. Table 1 shows the patients’ demographics, clinical and laboratory features and response to anakinra. Furthermore, patient 1 developed a life-threatening macrophage activation syndrome during the course of the disease, whereas patient 3 was refractory to methotrexate and etanercept.