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Kawasaki disease — SCE Rheumatology MCQ

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HardVasculitisKawasaki diseaseSCE Rheumatology

A child with Still disease develops persistent fever, confusion, platelets 58 × 10⁹/L, fibrinogen 1.2 g/L, triglycerides 3.8 mmol/L and ferritin 45,000 micrograms/L. What is the immediate priority?

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Correct answer: EBegin urgent specialist treatment for macrophage-activation syndrome

Unremitting fever, neurological change, cytopenia, falling fibrinogen, hypertriglyceridaemia and extreme ferritin indicate macrophage-activation syndrome. Treatment must begin urgently—usually high-dose glucocorticoid with phenotype-directed ciclosporin or IL-1 blockade—while infection and other triggers are investigated in parallel.

Reference: EULAR/PReS recommendations for Still disease across children and adults (Published November 2024): https://ard.bmj.com/content/83/12/1614; NICE TA685: anakinra for Still disease (Published March 2021): https://www.nice.org.uk/guidance/ta685/chapter/1-Recommendations