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Profile of inflammatory mediators in tonsils of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.
MedLine Citation:
PMID:  23877488     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
The purpose of this study was to analyze the levels of white blood cells and profile of proinflammatory Th1, Th2, Th17, and T regulatory tissue cytokines in the tonsils of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) patients to contribute to the pathophysiological understanding of the PFAPA syndrome. A cohort of PFAPA patients who had tonsillectomy during 2010 and 2011 was included and compared to control patients who had tonsillectomy for tonsillar hypertrophy. White blood cell counts were measured during flares in PFAPA patients and before tonsillectomy in the control group. Cytokine gene expression was analyzed in removed tonsils by real-time PCR. Nine PFAPA patients with a median age of 5.3 years (1.7-8 years) and 17 hypertrophic tonsils of patients with a median age of 4.8 years (2.3-8.4 years) participated in this study. Tonsillectomy was performed during afebrile period between PFAPA flares. Three of the nine patients had recurrent episodes of aphthous stomatitis without fever after tonsillectomy. Leukocyte and neutrophil counts were higher in PFAPA patients compared to controls (p < 0.05). Eosinophil counts were lower in PFAPA patients during flares (p = 0.006). IL-1β, TNF-α, TGF-β, IL-17, and IFN-γ levels were similar in the tonsils of patients and controls. IL-4 gene expression in the tonsils was lower in PFAPA patients compared to those of the controls (p = 0.04). Proinflammatory, effector, and regulatory cytokine gene expression in tonsil tissue of PFAPA children removed in a noninflammatory asymptomatic interval and in control patients were similar. However, IL-4 cytokine gene expression in the tonsils and peripheral blood eosinophils were lower in the PFAPA patients suggesting a potential pathogenesis pathway based on an inhibition of Th2 responses.
Authors:
Patricia M Valenzuela; Andrea Araya; Claudio I Pérez; Ximena Maul; Carolina Serrano; Constanza Beltrán; Paul R Harris; Eduardo Talesnik
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-7-23
Journal Detail:
Title:  Clinical rheumatology     Volume:  -     ISSN:  1434-9949     ISO Abbreviation:  Clin. Rheumatol.     Publication Date:  2013 Jul 
Date Detail:
Created Date:  2013-7-23     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8211469     Medline TA:  Clin Rheumatol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Division of Pediatrics, Facultad de Medicina, Pontificia Universidad Católica de Chile, Lira 85, 5th floor, Santiago, Chile, pvalenzu@med.puc.cl.
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