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Related Experiment Video

Updated: Apr 3, 2026

Whole-cell MALDI-TOF Mass Spectrometry is an Accurate and Rapid Method to Analyze Different Modes of Macrophage Activation
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Whole-cell MALDI-TOF Mass Spectrometry is an Accurate and Rapid Method to Analyze Different Modes of Macrophage Activation

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Macrophage Activation Syndrome.

Ethan S Sen1,2, Sarah L N Clarke1,2, Athimalaipet V Ramanan3

  • 1Department of Pediatric Rheumatology, Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol, BS2 8BJ, UK.

Indian Journal of Pediatrics
|September 25, 2015
PubMed
Summary
This summary is machine-generated.

Macrophage activation syndrome (MAS), a severe complication of rheumatic diseases, involves immune over-activation. Early recognition and high-dose corticosteroids are key, with biologics like anakinra showing promise for refractory cases.

Keywords:
Hemophagocytic lymphohistiocytosisHyperferritinemiaInterleukin-1Macrophage activation syndromeSystemic juvenile idiopathic arthritis

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Area of Science:

  • Rheumatology
  • Immunology
  • Pediatrics

Background:

  • Macrophage activation syndrome (MAS) is a life-threatening condition linked to rheumatic diseases like systemic juvenile idiopathic arthritis (sJIA).
  • It's characterized by immune dysregulation, leading to a cytokine storm and multi-organ impairment.
  • Diagnosis can be challenging due to similarities with sepsis or disease flares.

Purpose of the Study:

  • To outline the diagnostic criteria and management strategies for MAS in sJIA patients.
  • To review current treatment approaches and explore emerging therapies.

Main Methods:

  • International consensus criteria for MAS diagnosis in sJIA.
  • Review of clinical features, diagnostic markers, and treatment outcomes.
  • Analysis of current first-line therapies (corticosteroids, cyclosporine, IVIg) and novel biologic agents.

Main Results:

  • Key features include fever, organomegaly, cytopenias, and specific laboratory abnormalities (high ferritin, low fibrinogen).
  • High-dose corticosteroids are the primary treatment, with adjunctive therapies for non-responders.
  • Anakinra (IL-1 receptor antagonist) demonstrates potential effectiveness in refractory MAS.

Conclusions:

  • Early diagnosis of MAS is crucial for effective management.
  • While corticosteroids are standard, targeted biologic therapies are emerging as important options.
  • Further research is needed to optimize treatment for refractory MAS.