Objective: Adult onset Still's disease (AOSD) is a systemic disorder with an uncertain outcome at the time of diagnosis. The clinical response to nonsteroidal antiinflammatory drugs (NSAIDs) is often unsatisfactory in adult patients. Chronic use of steroids is frequently required, but may result in severe side effects. We report the results of an uncontrolled, unblinded trial of intravenous immunoglobulin (IVIG) in 7 patients with AOSD.
Methods: Seven consecutive patients unresponsive or poorly responsive to NSAIDs were enrolled. None of them had received steroids previously. AOSD was defined by the Yamaguchi criteria (J Rheumatol 1992; 19: 424). IVIG were administered every 4 weeks. NSAIDs were initially maintained. A positive response was defined by the disappearance of fever and arthritis within 2 weeks after the first IVIG infusion.
Results: Two patients failed to respond. Five patients initially considered to be good responders were given a total of 4 to 6 IVIG infusions. One of them relapsed at the time of the fourth IVIG infusion. The four others had a favourable clinical and biological course. At the time of evaluation 3 patients were symptom-free and off therapy, while one was still receiving low dose NSAIDs.
Conclusion: This short, uncontrolled, unblinded study suggests that IVIG might represent a potential alternative to classical steroid therapy in patients with AOSD refractory to NSAIDs. These preliminary results need to be confirmed, however, in a double-blind randomized study.