Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Apr 15;18(1):31.
doi: 10.1186/s12969-020-00424-x.

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

Gil Amarilyo  1 , Deborah Rothman  2 , Kalpana Manthiram  3 , Kathryn M Edwards  4 , Suzanne C Li  5 , Gary S Marshall  6 , Cagri Yildirim-Toruner  7 , Kathleen Haines  5 , Polly J Ferguson  8 , Geraldina Lionetti  9 , Julie Cherian  10 , Yongdong Zhao  11 , Patricia DeLaMora  12 , Grant Syverson  13 , Simona Nativ  14 , Marinka Twilt  15 , Ian C Michelow  16 , Yuriy Stepanovskiy  17 , Akaluck Thatayatikom  18 , Liora Harel  19 , Shoghik Akoghlanian  7 , Lori Tucker  20 , Mariana Correia Marques  21 , Hemalatha Srinivasalu  22 , Evan J Propst  23 , Greg R Licameli  21 , Fatma Dedeoglu  21 , Sivia Lapidus  5 ; CARRA PFAPA Consensus Treatment Plan Workgroup
Collaborators, Affiliations

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

Gil Amarilyo et al. Pediatr Rheumatol Online J. .

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.

Keywords: Consensus treatment plan; PFAPA; Periodic fever; Recurrent fever.

PubMed Disclaimer

Conflict of interest statement

There are no conflicts of interest.

Figures

Fig. 1
Fig. 1
Consensus treatment plans for PFAPA
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Førsvoll J, Kristoffersen EK, Øymar K. Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study. Acta Paediatr. 2013;102:187–192. doi: 10.1111/apa.12069. - DOI - PubMed
    1. Gattorno M, Hofer M, Federici S, Vanoni F, Bovis F, Aksentijevich I, Anton J, Arostegui JI, Barron K, Ben-Cherit E, Brogan PA, Cantarini L, Ceccherini I, De Benedetti F, Dedeoglu F, Demirkaya E, Frenkel J, Goldbach-Mansky R, Gul A, Hentgen V, Hoffman H, Kallinich T, Kone-Paut I, Kuemmerle-Deschner J, Lachmann HJ, Laxer RM, Livneh A, Obici L, Ozen S, Rowczenio D, Russo R, Shinar Y, Simon A, Toplak N, Touitou I, Uziel Y, van Gijn M, Foell D, Garassino C, Kastner D, Martini A, Sormani MP, Ruperto N, Eurofever Registry and the Paediatric Rheumatology International Trials Organisation (PRINTO) Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis. 2019;78(8):1025–1032. doi: 10.1136/annrheumdis-2019-215048. - DOI - PubMed
    1. Król P, Böhm M, Sula V, Dytrych P, Katra R, Nemcová D, et al. PFAPA syndrome: clinical characteristics and treatment outcomes in a large single-Centre cohort. Clin Exp Rheumatol. 2013;31:980–987. - PubMed
    1. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 1987;110:43–46. doi: 10.1016/S0022-3476(87)80285-8. - DOI - PubMed
    1. Padeh S, Brezniak N, Zemer D, Pras E, Livneh A, Langevitz P, et al. Periodic fever, aphthous stomatitis, pharyngitis and adeopathy syndrome: clinical characteristics and outcome. J Pediatr. 1999;135:98–101. doi: 10.1016/S0022-3476(99)70335-5. - DOI - PubMed

Publication types

MeSH terms