University of Oulu

Renko, M., Lantto, U., & Tapiainen, T. (2019). Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. Acta Paediatrica. https://doi.org/10.1111/apa.14792

Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome

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Author: Renko, Marjo1,2; Lantto, Ulla2,3; Tapiainen, Terhi2,4
Organizations: 1Department of Paediatrics, University of Eastern Finland and Kuopio University Hospital, Kuopio, Finland
2PEDEGO Research Unit, University of Oulu, Oulu, Finland
3Department of Otorhinolaryngology, Oulu University Hospital, Oulu, Finland
4Department of Children and Adolescents, Oulu University Hospital, Oulu, Finland
Format: article
Version: accepted version
Access: embargoed
Persistent link: http://urn.fi/urn:nbn:fi-fe2019091828609
Language: English
Published: John Wiley & Sons, 2019
Publish Date: 2020-03-22
Description:

Abstract

Aim: Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of a periodic fever in childhood. The exact pathogenesis and the aetiology of PFAPA are still unknown.

Methods: We conducted a non‐systematic review of published articles about PFAPA syndrome and summarised the evidence for diagnostic criteria and treatment options for PFAPA.

Results: The first proposed diagnostic criteria for PFAPA, in addition to periodic fever, included aphthous stomatitis, pharyngitis or cervical lymphadenitis in children younger than five years at the beginning of the symptoms. C‐reactive protein (CRP) levels and leucocyte counts increase in most patients during episodes. Recent research reveals that tonsillectomy provides an immediate and long‐lasting cure for PFAPA, even in the absence of classic criteria of aphthous stomatitis, pharyngitis or cervical adenitis and in children older than five years.

Conclusion: We suggest that PFAPA can be diagnosed in children with at least five regularly occurring fever episodes without any other explanation, even in the absence of aphthous stomatitis, pharyngitis or cervical lymphadenitis and also in children older than five years.

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Series: Acta pædiatrica
ISSN: 0803-5253
ISSN-E: 1651-2227
ISSN-L: 0803-5253
Volume: 108
Issue: 8
Pages: 1385 - 1392
DOI: 10.1111/apa.14792
OADOI: https://oadoi.org/10.1111/apa.14792
Type of Publication: A2 Review article in a scientific journal
Field of Science: 3124 Neurology and psychiatry
Subjects:
Funding: This work was supported by the Society for Pediatric Research and the Finnish Medical Foundation.
Copyright information: © 2019 Foundation Acta Pædiatrica. Published by John Wiley & Sons Ltd 2019. This is the peer reviewed version of the following article: Renko, M., Lantto, U., & Tapiainen, T. (2019). Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. Acta Paediatrica. https://doi.org/10.1111/apa.14792, which has been published in final form at https://doi.org/10.1111/apa.14792. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Self-Archiving.