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)

for Eurofever and the Paediatric Rheumatology International Trials Organisation (PRINTO)

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)27
Number of pages6
JournalPediatric Rheumatology
Volume16
Issue number1
DOIs
Publication statusPublished - Apr 18 2018

Fingerprint

Lymphadenitis
Stomatitis
Fever
Aphthous Stomatitis
Pharyngitis
Periodicity
Surveys and Questionnaires
Adrenal Cortex Hormones
Research Personnel
Pediatrics

Keywords

  • Child
  • Child, Preschool
  • Consensus
  • Delphi Technique
  • Diagnosis, Differential
  • Fever/complications
  • Humans
  • Lymphadenitis/complications
  • Pharyngitis/complications
  • Stomatitis, Aphthous/complications
  • Surveys and Questionnaires
  • Syndrome

Cite this

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). / for Eurofever and the Paediatric Rheumatology International Trials Organisation (PRINTO).

In: Pediatric Rheumatology, Vol. 16, No. 1, 18.04.2018, p. 27.

Research output: Contribution to journalArticle

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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.",
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author = "{for Eurofever and the Paediatric Rheumatology International Trials Organisation (PRINTO)} and Federica Vanoni and Silvia Federici and Jordi Ant{\'o}n and Barron, {Karyl S} and Paul Brogan and {De Benedetti}, Fabrizio and Fatma Dedeoglu and Erkan Demirkaya and Veronique Hentgen and Tilmann Kallinich and Ronald Laxer and Ricardo Russo and Natasa Toplak and Yosef Uziel and Alberto Martini and Nicolino Ruperto and Marco Gattorno and Michael Hofer",
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T1 - 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)

AU - for Eurofever and the Paediatric Rheumatology International Trials Organisation (PRINTO)

AU - Vanoni, Federica

AU - Federici, Silvia

AU - Antón, Jordi

AU - Barron, Karyl S

AU - Brogan, Paul

AU - De Benedetti, Fabrizio

AU - Dedeoglu, Fatma

AU - Demirkaya, Erkan

AU - Hentgen, Veronique

AU - Kallinich, Tilmann

AU - Laxer, Ronald

AU - Russo, Ricardo

AU - Toplak, Natasa

AU - Uziel, Yosef

AU - Martini, Alberto

AU - Ruperto, Nicolino

AU - Gattorno, Marco

AU - Hofer, Michael

PY - 2018/4/18

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N2 - 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.

AB - 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.

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KW - Consensus

KW - Delphi Technique

KW - Diagnosis, Differential

KW - Fever/complications

KW - Humans

KW - Lymphadenitis/complications

KW - Pharyngitis/complications

KW - Stomatitis, Aphthous/complications

KW - Surveys and Questionnaires

KW - Syndrome

U2 - 10.1186/s12969-018-0246-9

DO - 10.1186/s12969-018-0246-9

M3 - Article

C2 - 29669569

VL - 16

SP - 27

JO - Pediatric Rheumatology

JF - Pediatric Rheumatology

SN - 1546-0096

IS - 1

ER -