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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dc.contributor.authorAmarilyo, Gil
dc.contributor.authorRothman, Deborah
dc.contributor.authorManthiram, Kalpana
dc.contributor.authorEdwards, Kathryn M
dc.contributor.authorLi, Suzanne C
dc.contributor.authorMarshall, Gary S
dc.contributor.authorYildirim-Toruner, Cagri
dc.contributor.authorHaines, Kathleen
dc.contributor.authorFerguson, Polly J
dc.contributor.authorLionetti, Geraldina
dc.contributor.authorCherian, Julie
dc.contributor.authorZhao, Yongdong
dc.contributor.authorDeLaMora, Patricia
dc.contributor.authorSyverson, Grant
dc.contributor.authorNativ, Simona
dc.contributor.authorTwilt, Marinka
dc.contributor.authorMichelow, Ian C
dc.contributor.authorStepanovskiy, Yuriy
dc.contributor.authorThatayatikom, Akaluck
dc.contributor.authorHarel, Liora
dc.contributor.authorAkoghlanian, Shoghik
dc.contributor.authorTucker, Lori
dc.contributor.authorMarques, Mariana C
dc.contributor.authorSrinivasalu, Hemalatha
dc.contributor.authorPropst, Evan J
dc.contributor.authorLicameli, Greg R
dc.contributor.authorDedeoglu, Fatma
dc.contributor.authorLapidus, Sivia
dc.date.accessioned2020-04-19T00:03:29Z
dc.date.available2020-04-19T00:03:29Z
dc.date.issued2020-04-15
dc.date.updated2020-04-19T00:03:29Z
dc.description.abstractAbstract 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.
dc.identifier.citationPediatric Rheumatology. 2020 Apr 15;18(1):31
dc.identifier.doihttps://doi.org/10.1186/s12969-020-00424-x
dc.identifier.urihttp://hdl.handle.net/1880/111810
dc.identifier.urihttps://doi.org/10.11575/PRISM/44161
dc.language.rfc3066en
dc.rights.holderThe Author(s)
dc.titleConsensus 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
dc.typeJournal Article
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