Towards a new set of classification criteria for PFAPA syndrome

TY - JOUR

T1 - Towards a new set of classification criteria for PFAPA syndrome

AU - Vanoni, Federica

AU - Caorsi, Roberta

AU - Aeby, Sandra

AU - Cochard, Marie

AU - Antón, Jordi

AU - Berg, Stefan

AU - Brik, Riva

AU - Dolezalova, Pavla

AU - Koné-Paut, Isabelle

AU - Neven, Benedicte

AU - Ozen, Seza

AU - Pillet, Pascal

AU - Stojanov, Silvia

AU - Wouters, Carine

AU - Gattorno, Marco

AU - Hofer, Michaël

PY - 2018/9/21

Y1 - 2018/9/21

N2 - 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.

AB - 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.

KW - Consensus

KW - Delphi Technique

KW - Diagnosis, Differential

KW - Fever

KW - Hereditary Autoinflammatory Diseases

KW - Humans

KW - Lymphadenitis

KW - Pharyngitis

KW - Stomatitis, Aphthous

KW - Syndrome

KW - Journal Article

U2 - 10.1186/s12969-018-0277-2

DO - 10.1186/s12969-018-0277-2

M3 - SCORING: Journal article

C2 - 30241480

VL - 16

SP - 60

JO - PEDIATR RHEUMATOL

JF - PEDIATR RHEUMATOL

SN - 1546-0096

IS - 1

ER -