Towards a new set of classification criteria for PFAPA syndrome

Federica Vanoni,Silvia Stojanov,Isabelle Koné-Paut,Pascal Pillet,Sandra Aeby,Jordi Antón,Roberta Caorsi,Pavla Dolezalova,Marco Gattorno,Riva Brik,Benedicte Neven,Seza Ozen,Stefan Berg,Michaël Hofer,Marie Cochard,Carine Wouters

doi:10.1186/s12969-018-0277-2

Abstract

BackgroundDiagnosis 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.MethodsA 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.ResultsThe 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.ConclusionOur 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.

Highlights

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
Periodic Fever, Aphthous stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) syndrome is characterized by regularly recurrent fever flares of early onset, accompanied by pharyngitis, cervical lymphadenopathy and oral aphthous ulcers [1]
Vanoni et al Pediatric Rheumatology (2018) 16:60 syndrome with higher specificity. This project was conducted through several steps: 1) a preliminary Delphi survey among international pediatricians and rheumatologists; 2) a consensus conference of physicians with specific expertise in PFAPA to obtain a provisional new set of classification criteria; 3) a preliminary evaluation of the new set of criteria on a cohort of real PFAPA patients

Summary

Introduction

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. In 2008 we started an international collaborative effort, aimed at developing new classification criteria based on expert consensus and analysis of real patient data, to identify a new set of classification criteria for PFAPA. Vanoni et al Pediatric Rheumatology (2018) 16:60 syndrome with higher specificity This project was conducted through several steps: 1) a preliminary Delphi survey among international pediatricians and rheumatologists; 2) a consensus conference of physicians with specific expertise in PFAPA to obtain a provisional new set of classification criteria; 3) a preliminary evaluation of the new set of criteria on a cohort of real PFAPA patients

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