Most common autoinflammatory syndrome in pediatric population

Most common autoinflammatory syndrome in pediatric population

  • • Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA).
  • • Recurrent episodes lasting ∼5 days, occur predictably ∼every 28 days; children are well in between episodes.
  • • Onset typically under age 5; most cases resolve by adolescence.
  • • No genetic mutations or ethnic factors found to be associated with PFAPA.
  • • Excellent outcome, but large impact on quality of life (missed school, unneeded medical workup).
  • • Clinical diagnosis, and remains a diagnosis of exclusion.
  • • Treatment (depends upon severity):
    • • Nonsteroidal antiinflammatory drugs (NSAIDs)/Acetaminophen during flares.
    • • Prednisone 1 to 2 mg/kg × 1 dose at fever onset (can be repeated once after 24 hours if still febrile); highly effective abortive therapy for most patients, but flares may start to occur more frequently.
    • • Cimetidine and colchicine can be used for prophylaxis.
    • • Tonsillectomy can be effective if patients fail nonsurgical therapy.
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