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
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 pla...
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2020-04-01
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Series: | Pediatric Rheumatology Online Journal |
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Online Access: | http://link.springer.com/article/10.1186/s12969-020-00424-x |
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Article |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
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 Julie Cherian Yongdong Zhao Patricia DeLaMora Grant Syverson Simona Nativ Marinka Twilt Ian C. Michelow Yuriy Stepanovskiy Akaluck Thatayatikom Liora Harel Shoghik Akoghlanian Lori Tucker Mariana Correia Marques Hemalatha Srinivasalu Evan J. Propst Greg R. Licameli Fatma Dedeoglu Sivia Lapidus for the CARRA PFAPA Consensus Treatment Plan Workgroup |
spellingShingle |
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 Julie Cherian Yongdong Zhao Patricia DeLaMora Grant Syverson Simona Nativ Marinka Twilt Ian C. Michelow Yuriy Stepanovskiy Akaluck Thatayatikom Liora Harel Shoghik Akoghlanian Lori Tucker Mariana Correia Marques Hemalatha Srinivasalu Evan J. Propst Greg R. Licameli Fatma Dedeoglu Sivia Lapidus for the CARRA PFAPA Consensus Treatment Plan Workgroup 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 PFAPA Periodic fever Recurrent fever Consensus treatment plan |
author_facet |
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 Julie Cherian Yongdong Zhao Patricia DeLaMora Grant Syverson Simona Nativ Marinka Twilt Ian C. Michelow Yuriy Stepanovskiy Akaluck Thatayatikom Liora Harel Shoghik Akoghlanian Lori Tucker Mariana Correia Marques Hemalatha Srinivasalu Evan J. Propst Greg R. Licameli Fatma Dedeoglu Sivia Lapidus for the CARRA PFAPA Consensus Treatment Plan Workgroup |
author_sort |
Gil Amarilyo |
title |
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 |
title_short |
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 |
title_full |
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 |
title_fullStr |
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 |
title_full_unstemmed |
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 |
title_sort |
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 |
publisher |
BMC |
series |
Pediatric Rheumatology Online Journal |
issn |
1546-0096 |
publishDate |
2020-04-01 |
description |
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. |
topic |
PFAPA Periodic fever Recurrent fever Consensus treatment plan |
url |
http://link.springer.com/article/10.1186/s12969-020-00424-x |
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doaj-5882ea8313504d738cf58f01099ccccf2020-11-25T03:01:00ZengBMCPediatric Rheumatology Online Journal1546-00962020-04-011811710.1186/s12969-020-00424-xConsensus 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 groupGil Amarilyo0Deborah Rothman1Kalpana Manthiram2Kathryn M. Edwards3Suzanne C. Li4Gary S. Marshall5Cagri Yildirim-Toruner6Kathleen Haines7Polly J. Ferguson8Geraldina Lionetti9Julie Cherian10Yongdong Zhao11Patricia DeLaMora12Grant Syverson13Simona Nativ14Marinka Twilt15Ian C. Michelow16Yuriy Stepanovskiy17Akaluck Thatayatikom18Liora Harel19Shoghik Akoghlanian20Lori Tucker21Mariana Correia Marques22Hemalatha Srinivasalu23Evan J. Propst24Greg R. Licameli25Fatma Dedeoglu26Sivia Lapidus27for the CARRA PFAPA Consensus Treatment Plan WorkgroupPediatric Rheumatology Unit, Schneider Children’s Medical Center of Israel, Petach Tikva; Sackler Faculty of Medicine, Tel Aviv UniversityMassacusetts General Hospital for ChildrenNational Human Genome Research Institute, National Institutes of HealthVanderbilt University School of MedicineJoseph M Sanzari Children’s Hospital, Hackensack Meridian HealthDepartment of Pediatrics, University of LouisvilleNationwide Children’s HospitalJoseph M Sanzari Children’s Hospital, Hackensack Meridian HealthStead Family Department of Pediatrics, University of Iowa Carver College of MedicineUCSF Benioff Children’s Hospital OaklandStony Brook University HospitalSeattle Children’s Hospital, University of WashingtonWeill Cornell Medical CollegeMedical College of WisconsinGoryeb Children’s HospitalRheumatology, Department of Pediatrics, Alberta Children’s Hospital, University of CalgaryAlpert Medical School of Brown UniversityShupyk National Medical Academy of Postgraduate EducationDepartment of Pediatrics, University of FloridaPediatric Rheumatology Unit, Schneider Children’s Medical Center of Israel, Petach Tikva; Sackler Faculty of Medicine, Tel Aviv UniversityNationwide Children’s HospitalBC Children’s HospitalBoston Children’s Hospital, Harvard Medical SchoolChildren’s National Medical CenterHospital for Sick Children, University of TorontoBoston Children’s Hospital, Harvard Medical SchoolBoston Children’s Hospital, Harvard Medical SchoolJoseph M Sanzari Children’s Hospital, Hackensack Meridian HealthAbstract 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.http://link.springer.com/article/10.1186/s12969-020-00424-xPFAPAPeriodic feverRecurrent feverConsensus treatment plan |