CAPS — RACP Paediatrics MCQ

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An infant has chronic urticaria-like rash from birth, fever, progressive SNHL, chronic sterile meningitis, papilloedema. NLRP3 mutation. What is the diagnosis?

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Correct answer: C — CAPS

CAPS (CINCA/NOMID): IL-1-mediated autoinflammatory. Anti-IL-1 therapy (anakinra, canakinumab) dramatically effective.

Reference: RACP Paediatric Curriculum – Rheumatology