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Pediatr Rheumatol Online J. 2018 Sep 21;16(1):60. doi: 10.1186/s12969-018-0277-2.

Towards a new set of classification criteria for PFAPA syndrome.

Author information

1
Istituto Pediatrico della Svizzera Italiana, Ospedale San Giovanni, 6500, Bellinzona, Switzerland. federica.vanoni@eoc.ch.
2
Unité Romande d'Immuno-rhumatologie Pédiatrique, CHUV, University of Lausanne, Lausanne and HUG, Geneva, Switzerland. federica.vanoni@eoc.ch.
3
Istituto Giannina Gaslini, Clinica Pediatrica e Reumatologia, PRINTO, Genoa, Italy.
4
Unité Romande d'Immuno-rhumatologie Pédiatrique, CHUV, University of Lausanne, Lausanne and HUG, Geneva, Switzerland.
5
Pediatric Rheumatology, Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat, Barcelona, Spain.
6
Department of Pediatrics, The Queen Silvia Children's Hospital, Goteborg, Sweden.
7
Department of Pediatrics and Pediatric Rheumatology Service, Ruth Rappaport Children's Hospital, Rambam Medical Center, Haifa, Israel.
8
Department of Paediatrics and Adolescent Medicine, Paediatric Rheumatology Unit, General University Hospital and 1st Faculty of Medicine, Charles University in Prague, Praha, Czech Republic.
9
Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre APHP, University of Paris Sud -CEREMAIA, Paris, France.
10
Unité d'Immunologie-Hématologie et Rhumatologie Pédiatriques CHU Paris - Hôpital Necker-Enfants Malades, Paris, France.
11
Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey.
12
Service de pédiatrie médicale CHRU de Bordeaux, Bordeaux, France.
13
Department of Infectious Diseases and Immunology, Children's Hospital, University of Munich, Munich, Germany.
14
Department of Microbiology and Immunology, Laboratory Paediatric Immunology, UZ Leuven Hospital, Leuven, Belgium.

Abstract

BACKGROUND:

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.

METHODS:

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.

RESULTS:

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.

CONCLUSION:

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:

Autoinflammatory diseases; PFAPA

PMID:
30241480
PMCID:
PMC6151014
DOI:
10.1186/s12969-018-0277-2
[Indexed for MEDLINE]
Free PMC Article

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