Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group.
Adrenal Cortex Hormones
/ therapeutic use
Advisory Committees
Antipyretics
/ therapeutic use
Child
Child, Preschool
Cimetidine
/ therapeutic use
Colchicine
/ therapeutic use
Fever
/ physiopathology
Histamine H2 Antagonists
/ therapeutic use
Humans
Lymphadenitis
/ physiopathology
Neck
Pharyngitis
/ physiopathology
Stomatitis, Aphthous
/ physiopathology
Syndrome
Tonsillectomy
Tubulin Modulators
/ therapeutic use
Consensus treatment plan
PFAPA
Periodic fever
Recurrent fever
Journal
Pediatric rheumatology online journal
ISSN: 1546-0096
Titre abrégé: Pediatr Rheumatol Online J
Pays: England
ID NLM: 101248897
Informations de publication
Date de publication:
15 Apr 2020
15 Apr 2020
Historique:
received:
16
12
2019
accepted:
03
04
2020
entrez:
16
4
2020
pubmed:
16
4
2020
medline:
5
3
2021
Statut:
epublish
Résumé
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA. The CARRA PFAPA Working Group is composed of pediatric rheumatologists, infectious disease specialists, allergists/immunologists and otolaryngologists. An extensive literature review was conducted followed by a survey to assess physician practice patterns. This was followed by virtual and in-person meetings between 2014 and 2018. Nominal group technique (NGT) was employed to develop CTPs, as well as inclusion criteria for entry into future treatment studies, and response criteria. Consensus required 80% agreement. The PFAPA working group developed CTPs resulting in 4 different treatment arms: 1. Antipyretic, 2. Abortive (corticosteroids), 3. Prophylaxis (colchicine or cimetidine) and 4. Surgical (tonsillectomy). Consensus was obtained among CARRA members for those defining patient characteristics who qualify for participation in the CTP PFAPA study. The goal is for the CTPs developed by our group to lead to future comparative effectiveness studies that will generate evidence-driven therapeutic guidelines for this periodic inflammatory disease.
Sections du résumé
BACKGROUND
BACKGROUND
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strategies and a lack of evidence-based treatment guidelines. Consensus treatment plans (CTPs) are standardized treatment regimens that are derived based upon best available evidence and current treatment practices that are a way to enable comparative effectiveness studies to identify optimal therapy and are less costly to execute than randomized, double blind placebo controlled trials. The purpose of this project was to develop CTPs and response criteria for PFAPA.
METHODS
METHODS
The CARRA PFAPA Working Group is composed of pediatric rheumatologists, infectious disease specialists, allergists/immunologists and otolaryngologists. An extensive literature review was conducted followed by a survey to assess physician practice patterns. This was followed by virtual and in-person meetings between 2014 and 2018. Nominal group technique (NGT) was employed to develop CTPs, as well as inclusion criteria for entry into future treatment studies, and response criteria. Consensus required 80% agreement.
RESULTS
RESULTS
The PFAPA working group developed CTPs resulting in 4 different treatment arms: 1. Antipyretic, 2. Abortive (corticosteroids), 3. Prophylaxis (colchicine or cimetidine) and 4. Surgical (tonsillectomy). Consensus was obtained among CARRA members for those defining patient characteristics who qualify for participation in the CTP PFAPA study.
CONCLUSION
CONCLUSIONS
The goal is for the CTPs developed by our group to lead to future comparative effectiveness studies that will generate evidence-driven therapeutic guidelines for this periodic inflammatory disease.
Identifiants
pubmed: 32293478
doi: 10.1186/s12969-020-00424-x
pii: 10.1186/s12969-020-00424-x
pmc: PMC7157990
doi:
Substances chimiques
Adrenal Cortex Hormones
0
Antipyretics
0
Histamine H2 Antagonists
0
Tubulin Modulators
0
Cimetidine
80061L1WGD
Colchicine
SML2Y3J35T
Types de publication
Consensus Development Conference
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
31Investigateurs
Ronald Laxer
(R)
Lisa Imundo
(L)
Paul Tsoukas
(P)
Peter Wright
(P)
Kelly Brown
(K)
Rima Khasawneh
(R)
Rosie Scuccimarri
(R)
Evan Mulvihill
(E)
Meghan Aabo
(M)
Edwin Anderson
(E)
Leslie Abramson
(L)
Daniela Adelean
(D)
Danielle Dumez
(D)
Marla Guzman
(M)
Renee Pang
(R)
Ellen Go
(E)
Katalin Koranyi
(K)
Donald Goldsmith
(D)
Hanna Kim
(H)
Andrew Zeft
(A)
Rayfel Schnieder
(R)
Victoria Statler
(V)
Lauren Steele
(L)
Lori Broderick
(L)
Hal Hoffman
(H)
Sriharsha Cherukumilli Grevich
(SC)
Elizabeth Chalom
(E)
Michal Cidon
(M)
Robert Sundel
(R)
Nadine Saad
(N)
Deborah McCurdy
(D)
Grant Schulert
(G)
Ali Yalcindag
(A)
Eric Yen
(E)
Sara Stern
(S)
Karen Durrant
(K)
Yonatan Butbul
(Y)
Jonathan Hausmann
(J)
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