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Case Report

Open Access

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

  • BARBARA STANIMIROVIĆ1
  • STOJISLAV KONJEVIĆ1
  • BILJANA ĐURĐEVIĆ-BANJAC1
  • DARIO ĐUKIĆ1
  • DEJAN PEJIĆ2
  • JELENA MRĐA3

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

*Corresponding Author(s): BARBARA STANIMIROVIĆ E-mail: barbarastanimirovic@gmail.com

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Abstract

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.

Key words

macrophage activation syndrome, rheumatic diseases, haemophagocytic lym-phohistiocytosis

Cite And Share

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.

References

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