Macrophage activation syndrome in a child with systemic onset of juvenile idiopathic arthritis

Abstract

Macrophage activation syndrome (MAS) is a serious and potentially fatal complica­tion of various diseases, mainly systemic inflammatory connective tissue disorders, the most frequent being systemic juvenile idiopathic arthritis (sJIA). Its most important clinical and laboratory manifestations are non-remitting fever, hepatosplenomegaly, impaired liver function, cytopenia, hypoal­buminemia, hypertriglyceridemia, hypofibrinogenemia and hyperferritinemia. The prognosis for the syndrome is often poor, so a prompt diagnosis and the appropriate medical treatment are critical for survival. We report a case of an 8-year-old boy who developed MAS as a complication of sJIA. The boy presented with liver insufficiency, impaired coagulation, encephalopathy and respiratory failure and was treated in intensive care unit (ICU). He has achieved complete remission of the disease after therapy with pulse doses of corticosteroids and ciclosporin.