MACROPHAGE ACTIVATION SYNDROME AS A COMPLICATION OF SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS – CASE REPORT

  • Viktorija Kerin Department of Allergology, Rheumatology and clinical immunology, University Children's Hospital Ljubljana, University medical centre Ljubljana, Slovenia
  • Nataša Toplak Department of Allergology, Rheumatology and clinical immunology, University Children's Hospital Ljubljana, University medical centre Ljubljana, Slovenia
  • Vladan Rajić Department of haemato-oncology, University Children's Hospital Ljubljana, University medical centre Ljubljana, Slovenia
  • Tadej Avčin Department of Allergology, Rheumatology and clinical immunology, University Children's Hospital Ljubljana, University medical centre Ljubljana, Slovenia
Keywords: macrophage activation syndrome, systemic juvenile idiopathic arthritis, anti-IL1 therapy

Abstract

Abstract

Macrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (SJIA). MAS is characterized by systemic inflammation caused by excessive or uncontrolled release of proinflammatory cytokines (cytokine storm). The diagnostic hallmark are hemophagocytic macrophages, that could be present in bone marrow, liver, spleen or lymph nodes. Clinical features are similar to a flare of the underlying rheumatic disease which makes early recognition and choice of the appropriate treatment difficult. Diagnosis is made according to the preliminary diagnostic guidelines for MAS complicating SJIA.

We report a case of an 11 years old girl with MAS as an initial presentation of SJIA. She was successfully treated with high doses of glucocorticoid and cyclosporine. After discontinuation of glucocorticoid therapy she developed a new flare of the disease which was successfully treated with interleukin 1 blocking agent anakinra.

 

 

 

 

 

 

 

 

 

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Author Biography

Nataša Toplak, Department of Allergology, Rheumatology and clinical immunology, University Children's Hospital Ljubljana, University medical centre Ljubljana, Slovenia

Služba za alergologijo, revmatologijo in klinično imunologijo, Pediatrična klinika

Medicinska fakulteta, Katedra za npediatrijo, asist.dr.

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Published
2014-05-15
How to Cite
1.
Kerin V, Toplak N, Rajić V, Avčin T. MACROPHAGE ACTIVATION SYNDROME AS A COMPLICATION OF SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS – CASE REPORT. TEST ZdravVestn [Internet]. 15May2014 [cited 8May2024];83(4). Available from: http://vestnik-dev.szd.si/index.php/ZdravVest/article/view/75
Issue
Vol 83 No 4 (2014): April
Section
Case report, short scientific article


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