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Macrophage Activation Syndrome — RACP Paediatrics MCQ

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HardRheumatologyMacrophage Activation SyndromeRACP Paediatrics

A 7-year-old boy presents with high spiking quotidian fevers (typically >39°C, spiking once or twice daily and returning to normal or below normal), a salmon-coloured evanescent macular rash, arthritis, lymphadenopathy, and hepatosplenomegaly. Ferritin is 12,000 mcg/L. What is the most serious complication to monitor for?

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Correct answer: B — Macrophage activation syndrome (MAS)

Systemic JIA carries a significant risk of macrophage activation syndrome (MAS), a life-threatening condition characterised by an uncontrolled hyperinflammatory response. Features include very high ferritin (often >10,000), falling ESR (paradoxically), liver dysfunction, coagulopathy (DIC), cytopenias, and CNS dysfunction. Markedly elevated ferritin in systemic JIA should prompt close monitoring for MAS.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Systemic JIA and Macrophage Activation Syndrome