Objective : An attempt was made to identify characteristic
cytokine profiles to distinguish periodic
fever with
aphthous stomatitis,
pharyngitis and cervical
adenitis syndrome (PFAPAS) from recurrent
tonsillitis, of which clinical manifestations are similar to those of PFAPAS in children. Methods : Serum concentrations of
IL-6,
IL-4 and IFN-γ were measured during febrile episodes in pediatric patients. Results : The levels of
IL-6 during febrile episodes were markedly increased above the upper limit of normal ranges in patients with both PFAPAS and recurrent
tonsillitis, but there were no significant differences between groups. The levels of
IL-4 during febrile episodes in PFAPAS patients were significantly lower than those in recurrent
tonsillitis patients. The levels of IFN-γ during febrile episodes in PFAPAS patients were significantly higher than those in recurrent
tonsillitis patients. Conclusion : In pediatric patients with PFAPAS, despite an increase of
IL-6,
IL-4 was suppressed with a marked increase of IFN-γ during febrile episodes. On the contrary, in febrile pediatric patients with recurrent
tonsillitis, both
IL-6 and
IL-4, but not IFN-γ were increased. The characteristic
cytokine profiles of
IL-6,
IL-4 and IFN-γ can be used for differential diagnosis of PFAPAS from recurrent
tonsillitis in children in clinical ear, nose and throat (ENT) settings. J. Med. Invest. 68 : 38-41, February, 2021.