부산대학교 도서관

Abstract Macrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (SJIA). MAS is characterized by systemic inflammation caused by excessive or uncontrolled release of proinflammatory cytokines (cytokine storm). The diagnostic hallmark are hemophagocytic macrophages, that could be present in bone marrow, liver, spleen or lymph nodes. Clinical features are similar to a flare of the underlying rheumatic disease which makes early recognition and choice of the appropriate treatment difficult. Diagnosis is made according to the preliminary diagnostic guidelines for MAS complicating SJIA.We report a case of an 11 years old girl with MAS as an initial presentation of SJIA. She was successfully treated with high doses of glucocorticoid and cyclosporine. After discontinuation of glucocorticoid therapy she developed a new flare of the disease which was successfully treated with interleukin 1 blocking agent anakinra.