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 VanoniFollow
Silvia Federici
Jordi Antón
Karyl S Barron
Paul Brogan
Fabrizio De Benedetti
Fatma Dedeoglu
Erkan Demirkaya, Western UniversityFollow
Veronique Hentgen
Tilmann Kallinich
Ronald Laxer
Ricardo Russo
Natasa Toplak
Yosef Uziel
Alberto Martini
Nicolino Ruperto
Marco Gattorno
Michael Hofer

Document Type

Article

Publication Date

4-18-2018

Journal

Pediatric Rheumatology

Volume

16

Issue

1

First Page

27

Last Page

27

URL with Digital Object Identifier

https://doi.org/10.1186/s12969-018-0246-9

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.

Notes

Article originally published at Pediatric Rheumatology, Vol. 16(1).

https://doi.org/10.1186/s12969-018-0246-9