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Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome PFAPA work group
Journal article   Open access   Peer reviewed

Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome PFAPA work group

Gil Amarilyo, Deborah Rothman, Kalpana Manthiram, Kathryn M Edwards, Suzanne C Li, Gary S Marshall, Cagri Yildirim-Toruner, Kathleen Haines, Polly J Ferguson, Geraldina Lionetti, …
Pediatric rheumatology online journal, v 18(1)
15 Apr 2020
url
https://doi.org/10.1186/s12969-020-00424-xView
Published, Version of Record (VoR) Open

Abstract

Arthritis Canker sores Care and treatment Cimetidine Colchicine Communicable diseases Comparative analysis Evidence-based medicine Lymphadenitis Sore throat Stomatitis Strategic planning (Business)
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. Keywords: PFAPA, Periodic fever, Recurrent fever, Consensus treatment plan

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Collaboration types
Domestic collaboration
International collaboration
Web of Science research areas
Pediatrics
Rheumatology
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