Cytokine profile in PFAPA syndrome suggests continuous inflammation and reduced anti-inflammatory response
Silvia Stojanov1,*, Florian Hoffmann1,*, Anja Kéry1, Ellen D. Renner1,2, Dominik Hartl1, Peter Lohse3, Kristina Huss1, Peter Fraunberger3, James D. Malley4, Stephanie Zellerer1, Michael H. Albert1, Bernd H. Belohradsky1
European Cytokine Network, Vol.17, No.2, pp. 90-97, 2006
Abstract PFAPA syndrome is characterized by periodic episodes of high fever, aphthous stomatitis,
pharyngitis, and/or cervical adenitis. It is of unknown etiology and manifests usually before 5 years of age. We
determined serum and intracellular cytokine levels in six PFAPA patients (4 males, 2 females, mean age 8 years
(± 1.2 SEM), range 4-13) during the symptom-free period as well as 6-12 hours and 18-24 hours after fever onset.
Values were compared to age-matched, healthy controls. Febrile PFAPA attacks led to a significant increase in
IL-6 and IFN-γ serum concentrations compared to symptom-free periods and to… More >