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).

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License: CC BY 4.0
Serval ID
serval:BIB_4B4DD5396B40
Type
Article: article from journal or magazin.
Collection
Publications
Institution
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).
Journal
Pediatric rheumatology online journal
Author(s)
Vanoni F., Federici S., Antón J., Barron K.S., Brogan P., De Benedetti F., Dedeoglu F., Demirkaya E., Hentgen V., Kallinich T., Laxer R., Russo R., Toplak N., Uziel Y., Martini A., Ruperto N., Gattorno M., Hofer M.
Working group(s)
for Eurofever and the Paediatric Rheumatology International Trials Organisation (PRINTO)
ISSN
1546-0096 (Electronic)
ISSN-L
1546-0096
Publication state
Published
Issued date
18/04/2018
Peer-reviewed
Oui
Volume
16
Number
1
Pages
27
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
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.
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.
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.
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.
Keywords
Autoinflammatory diseases, Monogenic periodic fever, PFAPA
Pubmed
Web of science
Open Access
Yes
Create date
26/04/2018 18:26
Last modification date
20/08/2019 13:59
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