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Macrophage activation syndrome in children with systemic juvenile idiopathic arthritis: a retrospective study on 7 patients


macrophage activation syndrome (MAS) is life-threatening complication of rheumatic diseases and is most frequent seen in systemic juvenile idiopathic arthritis (sJIA). Prompt recognition and immediate therapy is life saving.


to review clinical and laboratory data of MAS in 7 children with sJIA.


clinical and laboratory data of 7 patients with MAS, treated in our hospital from January 2008 to December 2013, were analyzed retrospectively.


seven children (4 females, 3 males) were studied. Two children had incomplete MAS. The underlying disease was not identified in one child. MAS developed during the course of underlying disease (sJIA) in three children. Clinical manifestations at diagnosis included high persistent fever (7), skin rash (6), hepatosplenomegaly (7), lymphadenopathy (6), hemorrhages (5) and central nervous system dysfunction (6). Laboratory data included: high feritin>10 000 (7), cytopenia (7), abnormal liver function tests (7), hypoalbunaemia (7), hypertriglyceridemia (5), coagulopathy (5), decreased erythrocyte sedimentation rate (5). Macrophage hemophagocytosis were found in 4 bone marrow aspiration. Rota virus was isolated in stool in 3 children. MAS was reccurent in two children (perforin gene done, negative). Six children responded on immunosupressive therapy and are doing well, one child died.


MAS is rare but serious complication of systemic juvenile idiopathic arthritis in children. It is important to keep in mind suddenly clinical and laboratory disturbanses in children with JIA, to recognise and immediate treat MAS in order to decrease mortality.

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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.

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Dizdarevic, A.O., Dinarevic, S.M., Selmanovic, V. et al. Macrophage activation syndrome in children with systemic juvenile idiopathic arthritis: a retrospective study on 7 patients. Pediatr Rheumatol 12 (Suppl 1), P218 (2014).

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