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Systemic JIA — RACP Paediatrics MCQ

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ModerateIDSystemic JIARACP Paediatrics

A 9-year-old presents with fever, evanescent salmon-pink macular rash, and polyarthritis. The rash appears with fever spikes and fades when afebrile. There is lymphadenopathy and hepatosplenomegaly. Ferritin is markedly elevated (>10,000 µg/L). What is the most likely diagnosis?

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Correct answer: B — Juvenile idiopathic arthritis (systemic)

Quotidian (daily spiking) fever with an evanescent salmon-pink rash that appears with fever and fades between spikes, polyarthritis, lymphadenopathy, hepatosplenomegaly, and very high ferritin is systemic juvenile idiopathic arthritis (sJIA, formerly Still disease). Macrophage activation syndrome (MAS) is a life-threatening complication to monitor for. IL-1 or IL-6 blockade (anakinra, tocilizumab) is first-line biologic therapy.

Reference: RCH Melbourne – 2023 – JIA CPG; RACP Paediatric Curriculum – Rheumatology