ArticlePediatric rheumatology online journal2020
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.
Article in Pediatric rheumatology online journal, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
32 citing papers in PubMed, 1 synthesis or guideline pooled it, 88 citations in OpenAlex.
- Surgical versus medical management of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis): a systematic review and network meta-analysis.Pediatric rheumatology online journal · 2026Pooled it
- Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome: An Updated Review with Current Insights into Pathogenesis and Therapeutic Strategies.Children (Basel, Switzerland) · 2026Review
- Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome reactivated by the initiation of oral immunotherapy (OIT).Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2026Article
- Epidemiology and clinical course of PFAPA syndrome in the pediatric population-single center study.BMC pediatrics · 2026Article
- Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome: a conundrum still hard to solve.Pediatric research · 2026Article
- Perinatal and early childhood determinants of disease persistence in PFAPA.Pediatric research · 2026Article
- Periodic fever, aphthous stomatitis, pharyngitis, adenitis: clinical manifestations, response to steroids, and a practice of online diagnosis and follow-up.Frontiers in immunology · 2026Article
- Cytokine profiles and laboratory parameters as indicators to distinguish children with PFAPA and bacterial infection.Frontiers in immunology · 2026Article
- Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome in Children-From Pathogenesis to Treatment Strategies: A Comprehensive Review.Paediatric drugs · 2025Review
- Features Associated with Response to Tonsillectomy in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome in Children.The Journal of pediatrics · 2025Article
- Colchicine treatment in PFAPA: how long should we wait for the clinical response?European journal of pediatrics · 2025Article
- Clinical, Immunologic, and Genetic Characteristics in Patients With Syndrome of Undifferentiated Recurrent Fevers.Arthritis & rheumatology (Hoboken, N.J.) · 2025Article
- Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome: An Update.Children (Basel, Switzerland) · 2025Review
- Exploring factors for predicting colchicine responsiveness in children with PFAPA.European journal of pediatrics · 2024Article
- Inflammatory turmoil within: an exploration of autoinflammatory disease genetic underpinnings, clinical presentations, and therapeutic approaches.Advances in rheumatology (London, England) · 2024Review
- Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation.Pediatric rheumatology online journal · 2024Article
- Impact of early immunosuppression on pediatric liver transplant outcomes within 1 year.Journal of pediatric gastroenterology and nutrition · 2024Article
- Use of Streptococcus Salivarius K12 in a cohort of PFAPA patients.Frontiers in pediatrics · 2024Article
- The riddle of recurrent fever: a clinical approach to pediatric autoinflammatory diseases.Frontiers in pediatrics · 2024Review
- [Glycosylphosphatidylinositol biosynthesis deficiency 15 caused byZhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2023Article
Corrections and comments
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Authors and funding
29 authors at 16 institutions in 4 countries.
Funding
Abstract
backgroundPeriodic 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.
methodsThe 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.
resultsThe 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.
conclusionThe 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.
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Registered trials
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