Format

Send to

Choose Destination
Pediatr Rheumatol Online J. 2018 Apr 18;16(1):27. doi: 10.1186/s12969-018-0246-9.

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).

Author information

1
Department of Pediatrics, 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
Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona, Esplugues de Llobregat, Barcelona, Spain.
5
NIH-NIAID, Division of Intramural Research, Bethesda, MD, USA.
6
Department of Infection, Inflammation and Rheumatology, Great Ormond St Hospital, London, UK.
7
IRCCS Ospedale Pediatrico Bambino Gesù, Division of Rheumatology, Rome, Italy.
8
Division of Immunology, Rheumatology Program, Harvard Medical School Boston, Boston Children's Hospital, Boston, MA, USA.
9
Division of Paediatric Rheumatology, Western University and Children's Hospital LHSC, London, ON, Canada.
10
Hôpital A Mignot - Centre Hospitalier de Versailles, CEREMAIA, French reference center for autoinflammatory diseases and inflammatory amyloidosis, Le Chesnay (Paris), France.
11
Pediatric Pneumology and Immunology, Charité Universitätsmedizin Berlin, Berlin, Germany.
12
Departments of Pediatrics and Medicine, University of Toronto and The Hospital for Sick Children, Toronto, ON, Canada.
13
Servicio de Inmunología y Reumatología, Hospital de Pediatría Juan P. Garrahan, Buenos Aires, Argentina.
14
Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia.
15
Meir Medical Centre, Pediatric Rheumatology Unit, Department of Pediatrics, Kfar Saba and Sackler School of Medicine, Tel Aviv University, Tel Aviv-Yafo, Israel.
16
Istituto Giannina Gaslini, Direzione Scientifica, Genoa, Italy.
17
Unité Romande d'Immuno-rhumatologie Pédiatrique, CHUV, University of Lausanne, Lausanne, and HUG, Geneva, Switzerland.

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.

KEYWORDS:

Autoinflammatory diseases; Monogenic periodic fever; PFAPA

PMID:
29669569
PMCID:
PMC5907175
DOI:
10.1186/s12969-018-0246-9
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for BioMed Central Icon for PubMed Central
Loading ...
Support Center