Abstract
BackgroundThis study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease.MethodsA cohort of patients with a median age of 4.9 years experiencing 'typical PFAPA' episodes participated in this study. Blood cells and serum cytokines were analyzed by CBC analysis and multiplex ELISA.ResultsOscillations in the concentration of blood cells during the afebrile and febrile phases of typical PFAPA syndrome were observed; novel findings include increased monocytes and decreased eosinophils during a febrile episode and increased thrombocytes in the afebrile interval. Relatively modest levels of pro-inflammatory cytokines were present in sera. IFNγ-induced cytokine IP10/CXCL10 was increased after the onset of fever while T cell-associated cytokines IL7 and IL17 were suppressed during afebrile and febrile periods.ConclusionsIdentification of dysregulated blood cells and serum cytokines is an initial step towards the identification of biomarkers of PFAPA disease and/or players in disease pathogenesis. Future investigations are required to conclusively discern which mediators are associated specifically with PFAPA syndrome.
Highlights
This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease
The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome was originally described by Marshall et al in 1987 and the acronym, PFAPA, was coined two years later together with the diagnostic criteria [1,2]
The actual incidence of PFAPA syndrome in the pediatric population is not known but it is more common than the hereditary periodic fevers, except in populations with an ethnic origin
Summary
This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease. PFAPA syndrome is regarded as a non-hereditary disease of unknown etiology the clinical observation is that, in a small proportion of cases, one of the parents or a more distant relative had similar symptoms in childhood [3]. The actual incidence of PFAPA syndrome in the pediatric population is not known but it is more common than the hereditary periodic fevers, except in populations with an ethnic origin symptom-free interval [3,10] Tonsillectomy has been correlated with the resolution of the disease in a majority of cases [11,12,13,14].
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