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
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Published:2020-04-15
Issue:1
Volume:18
Page:
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ISSN:1546-0096
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Container-title:Pediatric Rheumatology
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language:en
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Short-container-title:Pediatr Rheumatol
Author:
Amarilyo Gil, ,Rothman Deborah,Manthiram Kalpana,Edwards Kathryn M.,Li Suzanne C.,Marshall Gary S.,Yildirim-Toruner Cagri,Haines Kathleen,Ferguson Polly J.,Lionetti Geraldina,Cherian Julie,Zhao Yongdong,DeLaMora Patricia,Syverson Grant,Nativ Simona,Twilt Marinka,Michelow Ian C.,Stepanovskiy Yuriy,Thatayatikom Akaluck,Harel Liora,Akoghlanian Shoghik,Tucker Lori,Marques Mariana Correia,Srinivasalu Hemalatha,Propst Evan J.,Licameli Greg R.,Dedeoglu Fatma,Lapidus Sivia
Abstract
Abstract
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
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
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
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.
Publisher
Springer Science and Business Media LLC
Subject
Immunology and Allergy,Rheumatology,Pediatrics, Perinatology, and Child Health
Reference30 articles.
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