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AOSD Raised Ferritin — RACP Adult Medicine MCQ

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HardRheumatologyAOSD Raised FerritinRACP Adult Medicine

A 30-year-old with adult-onset Still disease develops worsening fever, confusion, cytopenias, triglyceride elevation, falling fibrinogen and ferritin above 40,000 micrograms/L. What complication should be presumed?

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Correct answer: D — Macrophage activation syndrome complicating adult-onset Still disease

The best answer is “Macrophage activation syndrome complicating adult-onset Still disease”. Cytopenias, hypofibrinogenaemia, hypertriglyceridaemia, neurological dysfunction and extreme ferritin in Still disease indicate macrophage activation syndrome, a secondary haemophagocytic lymphohistiocytosis requiring urgent specialist immunosuppression and trigger evaluation. An uncomplicated flare more often produces leukocytosis and does not explain consumptive coagulation abnormalities.

Reference: Australian Rheumatology Association: Adult inflammatory disease resources: https://rheumatology.org.au/For-Patients/Adult-Medication-Information