Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome

• Main Authors: Brown Kelly L (Author), Wekell Per (Author), Osla Veronica (Author), Sundqvist Martina (Author), Sävman Karin (Author), Fasth Anders (Author), Karlsson Anna (Author), Berg Stefan (Author)
• Format: Book
• Published: BMC, 2010-09-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Summary

<p>Abstract</p> <p>Background</p> <p>This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous <b>s</b>tomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pathophysiological understanding of this pediatric disease.</p> <p>Methods</p> <p>A 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.</p> <p>Results</p> <p>Oscillations 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.</p> <p>Conclusions</p> <p>Identification 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.</p>

Item Description

10.1186/1471-2431-10-65
1471-2431