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Intravenous Anakinra for Treating Macrophage Activation Syndrome in Adult-Onset Still's Disease

Berivan Bitik1, Mustafa Şenturk2, Seda Kibaroglu3

  • 1Department of Internal Medicine, Division of Rheumatology, Baskent University, Ankara, Turkey.

European Journal of Case Reports in Internal Medicine
|November 11, 2024
PubMed
Abstract

Insights

Adult-onset Still's disease (AOSD) can lead to severe macrophage activation syndrome (MAS). Prompt treatment with anakinra, even intravenously, is crucial for managing neurological symptoms and improving outcomes in critical AOSD cases.

Area of Science:

  • Rheumatology
  • Internal Medicine
  • Critical Care Medicine

Background:

  • Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder.
  • Macrophage activation syndrome (MAS) is a life-threatening complication of AOSD.
  • MAS presents diagnostic and therapeutic challenges, particularly with multi-organ involvement.

Purpose of the Study:

  • To present a case of severe AOSD with MAS and neurological involvement.
  • To highlight the challenges in diagnosing and managing MAS in AOSD.
  • To emphasize the potential benefit of anakinra in severe MAS cases.

Main Methods:

  • Case report of a 32-year-old male with AOSD and suspected MAS.
  • Initial treatment with corticosteroids and methotrexate, followed by intravenous methylprednisolone and immunoglobulin therapy.
  • Initiation of anakinra infusion due to suspected MAS and neurological deterioration.

Main Results:

  • The patient experienced altered consciousness and brainstem involvement, indicative of severe MAS.
  • Intravenous anakinra infusion led to significant clinical improvement.
  • This suggests anakinra may be effective in severe MAS with neurological complications.

Conclusions:

  • Early recognition and aggressive treatment are vital for managing severe AOSD complications like MAS.
  • Treatment delays for MAS, especially with neurological symptoms, can be detrimental.
  • Intravenous anakinra shows promise as a therapeutic option for severe MAS in AOSD.
Keywords:
Intravenous anakinraadult-onset Still’s diseasemacrophage activation syndrome

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