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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.
Pediatr Rheumatol Online J. 2020 Apr 15; 18(1):31.PR

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.

Authors+Show Affiliations

Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikva; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel. gamarilyo@clalit.org.il.Massacusetts General Hospital for Children, Boston, MA, USA.National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA.Vanderbilt University School of Medicine, Nashville, TN, USA.Joseph M Sanzari Children's Hospital, Hackensack Meridian Health, Hackensack, NJ, USA.Department of Pediatrics, University of Louisville, Louisville, KY, USA.Nationwide Children's Hospital, Columbus, OH, USA.Joseph M Sanzari Children's Hospital, Hackensack Meridian Health, Hackensack, NJ, USA.Stead Family Department of Pediatrics, University of Iowa Carver College of Medicine, Iowa City, IA, USA.UCSF Benioff Children's Hospital Oakland, Oakland, CA, USA.Stony Brook University Hospital, Stony Brook, NY, USA.Seattle Children's Hospital, University of Washington, Seattle, WA, USA.Weill Cornell Medical College, New York, NY, USA.Medical College of Wisconsin, Wauwatosa, WI, USA.Goryeb Children's Hospital, Morristown, NJ, USA.Rheumatology, Department of Pediatrics, Alberta Children's Hospital, University of Calgary, Calgary, Alberta, Canada.Alpert Medical School of Brown University, Providence, RI, USA.Shupyk National Medical Academy of Postgraduate Education, Kyiv, Ukraine.Department of Pediatrics, University of Florida, Gainesville, FL, USA.Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikva; Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.Nationwide Children's Hospital, Columbus, OH, USA.BC Children's Hospital, Vancouver, BC, Canada.Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.Children's National Medical Center, Washington, DC, USA.Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.Joseph M Sanzari Children's Hospital, Hackensack Meridian Health, Hackensack, NJ, USA.No affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

32293478

Citation

Amarilyo, Gil, et al. "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." Pediatric Rheumatology Online Journal, vol. 18, no. 1, 2020, p. 31.
Amarilyo G, Rothman D, Manthiram K, et al. 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. Pediatr Rheumatol Online J. 2020;18(1):31.
Amarilyo, G., Rothman, D., Manthiram, K., Edwards, K. M., Li, S. C., Marshall, G. S., Yildirim-Toruner, C., Haines, K., Ferguson, P. J., Lionetti, G., Cherian, J., Zhao, Y., DeLaMora, P., Syverson, G., Nativ, S., Twilt, M., Michelow, I. C., Stepanovskiy, Y., Thatayatikom, A., ... Lapidus, S. (2020). 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. Pediatric Rheumatology Online Journal, 18(1), 31. https://doi.org/10.1186/s12969-020-00424-x
Amarilyo G, et al. 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. Pediatr Rheumatol Online J. 2020 Apr 15;18(1):31. PubMed PMID: 32293478.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - 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. AU - Amarilyo,Gil, AU - Rothman,Deborah, AU - Manthiram,Kalpana, AU - Edwards,Kathryn M, AU - Li,Suzanne C, AU - Marshall,Gary S, AU - Yildirim-Toruner,Cagri, AU - Haines,Kathleen, AU - Ferguson,Polly J, AU - Lionetti,Geraldina, AU - Cherian,Julie, AU - Zhao,Yongdong, AU - DeLaMora,Patricia, AU - Syverson,Grant, AU - Nativ,Simona, AU - Twilt,Marinka, AU - Michelow,Ian C, AU - Stepanovskiy,Yuriy, AU - Thatayatikom,Akaluck, AU - Harel,Liora, AU - Akoghlanian,Shoghik, AU - Tucker,Lori, AU - Marques,Mariana Correia, AU - Srinivasalu,Hemalatha, AU - Propst,Evan J, AU - Licameli,Greg R, AU - Dedeoglu,Fatma, AU - Lapidus,Sivia, AU - ,, Y1 - 2020/04/15/ PY - 2019/12/16/received PY - 2020/04/03/accepted PY - 2020/4/16/entrez PY - 2020/4/16/pubmed PY - 2020/4/16/medline KW - Consensus treatment plan KW - PFAPA KW - Periodic fever KW - Recurrent fever SP - 31 EP - 31 JF - Pediatric rheumatology online journal JO - Pediatr Rheumatol Online J VL - 18 IS - 1 N2 - 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. SN - 1546-0096 UR - https://www.unboundmedicine.com/medline/citation/32293478/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 L2 - https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-020-00424-x DB - PRIME DP - Unbound Medicine ER -
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