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

Macrophage activation syndrome (MAS) is a serious and potentially fatal complica-tion of various diseases, mainly systemic in-flammatory connective tissue disorders, the most frequent being systemic juvenile idi-opathic arthritis (sJIA). Its most important clinical and laboratory manifestations are non-remitting fever, hepatosplenomegaly, impaired liver function, cytopenia, hypoal-buminemia, hypertriglyceridemia, hypofi-brinogenemia 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 in-tensive care unit (ICU). He has achieved complete remission of the disease after ther-apy with pulse doses of corticosteroids and ciclosporin.

macrophage activation syndrome, rheumatic diseases, haemophagocytic lym-phohistiocytosis

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