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Systemic JIA with MAS concern — SCE Rheumatology MCQ

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ModeratePaediatric rheumatologySystemic JIA with MAS concernSCE Rheumatology

A 9-year-old boy with systemic JIA becomes acutely unwell with persistent fever. Examination shows he has hepatomegaly and bruising. Investigations show ferritin is 18000 micrograms/L, platelets are falling and fibrinogen is low. What is the most appropriate management?

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Correct answer: A — Urgent treatment for macrophage activation syndrome

Falling platelets, low fibrinogen and very high ferritin in systemic JIA suggest macrophage activation syndrome and require urgent treatment. NSAID escalation alone is unsafe when the child is systemically deteriorating. MAS is a life-threatening hyperinflammatory complication and may occur even when arthritis is not prominent.

Reference: BSR juvenile idiopathic arthritis guideline; NICE CKS; BNF