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Macrophage Activation Syndrome — SCE Rheumatology MCQ

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HardRheumatology EmergenciesMacrophage Activation SyndromeSCE Rheumatology

An adult with Still disease develops persistent fever, cytopenias, ferritin 45,000 micrograms/L, high triglycerides, low fibrinogen and hepatitis. What complication should be treated urgently?

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Correct answer: B — Macrophage activation syndrome with haemophagocytic inflammation

The best answer is “Macrophage activation syndrome with haemophagocytic inflammation”. Fever, cytopenias, extreme ferritin, hypertriglyceridaemia, hypofibrinogenaemia and liver injury are classic MAS features and require urgent specialist treatment while infection is assessed. The pattern exceeds an uncomplicated flare, does not indicate Kawasaki disease or iron overload, and high ferritin does not exclude sepsis.

Reference: BSR adult-onset Still disease guideline: https://academic.oup.com/rheumatology/article/63/11/2993/7706976