Systemic JIA — RACP Paediatrics MCQ

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ModerateRheumatologySystemic JIARACP Paediatrics

A 10-year-old boy presents with fever for 2 weeks, salmon-pink evanescent rash, arthralgia, hepatosplenomegaly, lymphadenopathy, serositis, and markedly elevated ferritin (5000 mcg/L). ANA and RF are negative. What is the diagnosis?

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Correct answer: D — Systemic JIA (Still disease)

Systemic JIA: quotidian (daily spiking) fever, evanescent salmon-pink rash, arthritis/arthralgia, lymphadenopathy, hepatosplenomegaly, and serositis. Markedly elevated ferritin, CRP, and ESR. Complication: macrophage activation syndrome (MAS). Treatment includes NSAIDs, corticosteroids, and biologics (IL-1 or IL-6 inhibitors: anakinra, tocilizumab).

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Systemic JIA