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sJIA – MAS monitoring — RACP Paediatrics MCQ

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HardIDsJIA – MAS monitoringRACP Paediatrics

A 2-year-old presents with 3 weeks of daily spiking quotidian fever, evanescent salmon-pink rash, polyarthritis, lymphadenopathy, hepatosplenomegaly, and markedly elevated ferritin (>10,000). What is the most likely JIA subtype and what life-threatening complication must be monitored?

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Correct answer: D — JIA – systemic (sJIA)

Systemic JIA: quotidian fever + evanescent rash + polyarthritis + systemic features + very high ferritin. The life-threatening complication to monitor is macrophage activation syndrome (MAS): falling ferritin/ESR paradox, rising LDH/AST, falling fibrinogen/platelets, coagulopathy. MAS requires urgent immunosuppression.

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