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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
*Corresponding Author(s): BARBARA STANIMIROVIĆ E-mail: barbarastanimirovic@gmail.com
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.
1. Cassidy JT, Petty RE, Laxer RM, Lindsley CB. Textbook of pediatric rheumatology: macrophage activation syndrome,45, 6th ed. Elsevier Saunders; 2011. p. 674–81.
2. Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child 2001;85:421-6.
3. Ravelli A. Macrophage activation syndrome. Curr Opin Rheumatol. 2002;14:548-52.
4. Ravelli A, Magni-Manzoni A, Pistorio A, Besana C, Foti T,Ruperto N, et al. Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr. 2005;146:598–604.
5. Emmenegger U, Reimers A, Frey U, et al. Reactive macrophage activation syndrome: a simple screening strategy and its potential in early treatment initiation. Swiss Med Wkly 2002; 132: 230-236.
6. Weitzman S. Approach to hemophagocytic syndromes. AmSoc Hematol. 2011;1:178–83.9
7. Stephan JL, Kone-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM. Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology 2001; 40: 1285-1292.
8. Ravelli A, Caria MC, Buratti S, Mulattia C, Temporini F, MartiniA. Methotrexate as a possible trigger of macrophage activation syndrome in systemic juvenile idiopathic arthritis. J Rheumatol. 2001;28:865-7
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