Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) syndrome: analysis of patients from two geographic areas.

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) syndrome: analysis of patients from two geographic areas.

Arthritis Care Res (Hoboken). 2016 Apr 5;

Authors: Batu ED, Eroğlu FK, Tsoukas P, Hausmann JS, Bilginer Y, Kenna MA, Licameli GR, Fuhlbrigge RC, Özen S, Dedeoğlu F

Abstract
OBJECTIVE: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a periodic fever syndrome of childhood with an unknown etiology. Our aims were to compare the features between PFAPA patients from Turkey and the USA, and patients with and without MEFV variants, and to test the performance of the Eurofever criteria in excluding other autoinflammatory diseases.
METHODS: Seventy-one children with PFAPA followed in Hacettepe University, Ankara, Turkey and 60 patients in Boston Children's Hospital, Boston, USA were enrolled. MEFV gene variant analysis was performed in 56 patients with Sanger sequencing.
RESULTS: In patients from Turkey, symptom onset was at a younger age, fever attacks was of shorter duration, pharyngitis was more frequent while cervical adenitis, headache, and nausea/vomiting were less frequent during attacks when compared to patients from the USA (p˂0.05). More patients from the Turkey cohort were classified in the familial Mediterranean fever (FMF) group according to the Eurofever criteria than patients from the USA (66.2% versus 10%; p˂0.001). Two patients were diagnosed with FMF after MEFV analysis. Twenty-one (37.5%) had a single MEFV variant. No significant differences in phenotype were found between patients with and without MEFV variants.
CONCLUSION: The differences between patients from Turkey and USA cohorts may be due to epigenetic or environmental factors. In addition, the Eurofever FMF criteria may perform better in certain areas if the weight of ethnic origin parameter or cut-off values are modified. This article is protected by copyright. All rights reserved.

PMID: 27059542 [PubMed - as supplied by publisher]

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