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.
