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PFAPA syndrome — RACP Paediatrics MCQ

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ModerateIDPFAPA syndromeRACP Paediatrics

A 3-year-old has recurrent episodes of high fever (40°C) lasting 3-5 days occurring every 4-6 weeks. During episodes there is aphthous stomatitis, pharyngitis, and cervical lymphadenopathy. Between episodes the child is completely well and growing normally. Inflammatory markers normalise between episodes. What is the most likely diagnosis?

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Correct answer: B — PFAPA syndrome

The classic tetrad of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common periodic fever syndrome in children. Episodes are remarkably predictable in timing and duration. A single dose of prednisolone (1 mg/kg) dramatically aborts episodes (diagnostic and therapeutic). Tonsillectomy may be curative. The condition is self-limiting.

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