P03-004 - Production of proinflammatory cytokines in PFAPA

Introduction The PFAPA syndrome, associated with aphthous stomatitis, pharyngitis and cervical adenitis which is characterized by monthly flare-ups of fever is quite common in early childhood. Affected children experience their first attack before the age of 5, with fever episodes usually abating in adolescence or young adulthood. Usually a single administration of oral corticosteroids aborts attacks. Tonsillectomy is successful in the prevention of recurrence of further episodes. To date, the cause of this syndrome is unknown. Several studies demonstrated increased serum levels of proinflammatory cytokines(interleukin 6, interleukin 1b) in these patients during the attacks.