Periodic Fever with Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome, an Autoinflammatory Disease Treated with Tonsillectomy

Abstract

We report a case of PFAPA (Periodic fever with aphthous stomatitis, pharyngitis and cervical adenitis). A 5-year-old boy admitted for idiopathic fever 12 times within 8-months. Fever of >38°C occurred for 5 days at 10-to-20-days intervals. Antibiotics failed to decrease the fever. PFAPA was diagnosed when the boy was found to be febrile with aphthous stomatitis. Steroid administration decreased the fever, but not its recurrence. Following tonsillectomy, however, the boy had fever only once 2 weeks after surgery in the 12 months since. PFAPA etiology is unknown, but recent reports suggest irregular pro-inflammatory cytokine production. Serum IL-6, TNF-α and IFN-γ concentration increased during febrile attack in our case.