Anakinra treatment in macrophage activation syndrome: a single center experience and systemic review of literature

  • Hafize Emine Sönmez
  • Selcan Demir
  • Yelda Bilginer
  • Seza Özen
Original Article


Our aim was to report our experiences of pediatric macrophage activation syndrome (MAS) patients treated with anakinra and to review previous studies reporting anakinra treatment in pediatric MAS patients associated with systemic juvenile idiopathic arthritis (sJIA) or autoinflammatory diseases (AIDs). The study group consisted of pediatric MAS patients due to sJIA or AIDs, followed up in the Pediatric Rheumatology Unit of Hacettepe University between January 2015 and January 2017 and treated with anakinra (anti-IL1). We conducted a systematic review of the published literature involving pediatric MAS patients associated with sJIA or AIDs, treated with anakinra. Thirteen sJIA patients and two AIDs patients were included the study. Nineteen MAS episodes were observed in 15 patients. Anakinra (2 mg/kg/day) was started in with a median 1 day after admission. Clinical symptoms resolved, and laboratory findings normalized within median (minimum–maximum) 2 (1–4) and 6 (4–9) days, respectively after the introduction of anakinra. Steroid treatment was stopped in a median of 10 (4–13) weeks after the initiation of anakinra treatment. Patients were followed up for a median of 13 (6–24) months. Two patients developed recurrent MAS episodes when the anakinra dose was reduced, while the other patients achieved remission. In the literature review, we identified nine articles, describing 35 pediatric MAS patients associated with sJIA or AIDs and treated with anakinra. Except for two, all the patients reached remission. Our study and systematic literature review may help to improve the knowledge on the role of anakinra treatment in the management of MAS.


Anakinra Auto-inflammatory syndromes Juvenile idiopathic arthritis Macrophage activation syndrome 


Authorship contributions

Hafize Emine Sönmez conceptualized and designed the study, carried out the initial analyses, and approved the final manuscript as submitted. Selcan Demir and Yelda Bilginer helped designing the study, collected the data, drafted the initial manuscript, and approved the final manuscript as submitted. Seza Özen coordinated and supervised data collection, reviewed and revised the manuscript, and approved the final manuscript as submitted. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Compliance with ethical standards



Supplementary material

10067_2018_4095_MOESM1_ESM.docx (13 kb)
ESM 1 (DOCX 12 kb).


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Copyright information

© International League of Associations for Rheumatology (ILAR) 2018

Authors and Affiliations

  • Hafize Emine Sönmez
    • 1
  • Selcan Demir
    • 1
  • Yelda Bilginer
    • 1
  • Seza Özen
    • 1
  1. 1.Department of Pediatrics, Division of Rheumatology, Faculty of MedicineHacettepe UniversityAnkaraTurkey

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