Macrophage Activation Syndrome Successfully Treated with Dexamethasone Palmitate

Abstract

We report a case of macrophage activation syndrome (MAS) that was treated successfully with a liposteroid, dexamethasone palmitate. The patient was a 5-year-old girl who had developed systemic-onset juvenile idiopathic arthritis at the age of 3 years and 8 months, and suffered a second episode of MAS with high fever, vomiting, diarrhea and consciousness disturbance. Although her condition was complicated by disseminated intravascular coagulation, cardiac failure and renal insufficiency, administration of dexamethasone palmitate (10 mg/m²/day) promptly relieved her symptoms. Because rapidly fatal cases of MAS have been reported, prompt first-line treatment for MAS is important. In some cases of MAS with renal insufficiency where use of cyclosporine A is difficult, dexamethasone palmitate might be added as a first-line treatment of MAS.