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Pediatric Rheumatology
Published in: Pediatric Rheumatology 1/2018

Open Access 01-12-2018 | Research article

An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA)

Authors: Federica Vanoni, Silvia Federici, Jordi Antón, Karyl S. Barron, Paul Brogan, Fabrizio De Benedetti, Fatma Dedeoglu, Erkan Demirkaya, Veronique Hentgen, Tilmann Kallinich, Ronald Laxer, Ricardo Russo, Natasa Toplak, Yosef Uziel, Alberto Martini, Nicolino Ruperto, Marco Gattorno, Michael Hofer, for Eurofever and the Paediatric Rheumatology International Trials Organisation (PRINTO)

Published in: Pediatric Rheumatology | Issue 1/2018

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Abstract

Background

Diagnosis of Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is currently based on a set of criteria proposed in 1999 modified from Marshall’s criteria. Nevertheless no validated evidence based set of classification criteria for PFAPA has been established so far. The aim of this study was to identify candidate classification criteria PFAPA syndrome using international consensus formation through a Delphi questionnaire survey.

Methods

A first open-ended questionnaire was sent to adult and pediatric clinicians/researchers, asking to identify the variables thought most likely to be helpful and relevant for the diagnosis of PFAPA. In a second survey, respondents were asked to select, from the list of variables coming from the first survey, the 10 features that they felt were most important, and to rank them in descending order from most important to least important.

Results

The response rate to the first and second Delphi was respectively 109/124 (88%) and 141/162 (87%). The number of participants that completed the first and second Delphi was 69/124 (56%) and 110/162 (68%). From the first Delphi we obtained a list of 92 variables, of which 62 were selected in the second Delphi. Variables reaching the top five position of the rank were regular periodicity, aphthous stomatitis, response to corticosteroids, cervical adenitis, and well-being between flares.

Conclusion

Our process led to identification of features that were felt to be the most important as candidate classification criteria for PFAPA by a large sample of international rheumatologists. The performance of these items will be tested further in the next phase of the study, through analysis of real patient data.
Appendix
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Metadata
Title
An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA)
Authors
Federica Vanoni
Silvia Federici
Jordi Antón
Karyl S. Barron
Paul Brogan
Fabrizio De Benedetti
Fatma Dedeoglu
Erkan Demirkaya
Veronique Hentgen
Tilmann Kallinich
Ronald Laxer
Ricardo Russo
Natasa Toplak
Yosef Uziel
Alberto Martini
Nicolino Ruperto
Marco Gattorno
Michael Hofer
for Eurofever and the Paediatric Rheumatology International Trials Organisation (PRINTO)
Publication date
01-12-2018
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue 1/2018
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/s12969-018-0246-9

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