Towards a new set of classification criteria for PFAPA syndrome.

Details

Ressource 1Download: 30241480_BIB_E7FDDC57704D.pdf (521.13 [Ko])
State: Public
Version: Final published version
License: CC BY 4.0
Serval ID
serval:BIB_E7FDDC57704D
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Towards a new set of classification criteria for PFAPA syndrome.
Journal
Pediatric rheumatology online journal
Author(s)
Vanoni F., Caorsi R., Aeby S., Cochard M., Antón J., Berg S., Brik R., Dolezalova P., Koné-Paut I., Neven B., Ozen S., Pillet P., Stojanov S., Wouters C., Gattorno M., Hofer M.
ISSN
1546-0096 (Electronic)
ISSN-L
1546-0096
Publication state
Published
Issued date
21/09/2018
Peer-reviewed
Oui
Volume
16
Number
1
Pages
60
Language
english
Notes
Publication types: Journal Article
Publication Status: epublish
Abstract
Diagnosis of Periodic Fever, Aphthous stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) syndrome is currently based on the modified Marshall's criteria, but no validated evidence based classification criteria for PFAPA has been established so far.
A multistep process, based on the Delphi and Nominal Group Technique was conducted. After 2 rounds of e-mail Delphi survey involving 21 experts in autoinflammation we obtained a list of variables that were discussed in an International Consensus Conference. Variables reaching the 80% of consensus between participants were included in the new classification criteria. In the second phase the new classification criteria and the modified Marshall's criteria were applied on a cohort of 80 pediatric PFAPA patients to compare their performance.
The Delphi Survey was sent to 22 participants, 21 accepted to participate. Thirty variables were obtained from the survey and have been discussed at the Consensus Conference. Through the Nominal Group Technique we obtained a new set of classification criteria. These criteria were more restrictive in respect to the modified Marshall's criteria when applied on our cohort of patients.
Our work led us to identify a new set of classification criteria for PFAPA syndrome, but they resulted to be too restrictive to be applied in daily clinical practice for the diagnosis of PFAPA.
Keywords
Consensus, Delphi Technique, Diagnosis, Differential, Fever/complications, Hereditary Autoinflammatory Diseases/classification, Hereditary Autoinflammatory Diseases/diagnosis, Humans, Lymphadenitis/complications, Pharyngitis/complications, Stomatitis, Aphthous/complications, Syndrome, Autoinflammatory diseases, PFAPA
Pubmed
Web of science
Open Access
Yes
Create date
15/10/2018 16:57
Last modification date
20/08/2019 16:10
Usage data