Macrophage activation syndrome in a child with systemic onset of juvenile idiopathic arthritis
1Clinic of Paediatrics, University Hospital Clinical Centre, Banja Luka, Bosnia and Herzegovina
2General Hospital Gradiška, Gradiška, Bosnia and Herzegovina
3Clinic of Internal Medicine, University Hospital, Clinical Centre, Banja Luka, Bosnia and Herzegovina
DOI: 10.22514/SV101.062015.15 Vol.10,Issue S1,June 2015 pp.55-57
Published: 22 June 2015
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
BARBARA STANIMIROVIĆ,STOJISLAV KONJEVIĆ,BILJANA ĐURĐEVIĆ-BANJAC,DARIO ĐUKIĆ,DEJAN PEJIĆ,JELENA MRĐA. Macrophage activation syndrome in a child with systemic onset of juvenile idiopathic arthritis. Signa Vitae. 2015. 10(S1);55-57.
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