Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review

Alicia Wang; Kalpana Manthiram; Fatma Dedeoglu; Greg R Licameli

doi:10.1016/j.wjorl.2021.05.004

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review

World J Otorhinolaryngol Head Neck Surg. 2021 Jun 27;7(3):166-173. doi: 10.1016/j.wjorl.2021.05.004. eCollection 2021 Jul.

Authors

Alicia Wang¹, Kalpana Manthiram², Fatma Dedeoglu^{3

4}, Greg R Licameli^{1

4}

Affiliations

¹ Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, MA, USA.
² National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA.
³ Division of Immunology, Boston Children's Hospital, Boston, MA, USA.
⁴ Harvard Medical School, Boston, MA, USA.

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever condition in children, with most cases appearing by the age of 5. Although PFAPA is generally a self-limited condition, it can have a major impact on a child's quality of life, as well as that of their family. Recent research has continued to shed light on the genetic and immunologic factors that play a role in the pathogenesis of PFAPA. There also exists significant heterogeneity in treatment strategies, and progress has been made to develop evidence-based management strategies and establish a standard of care. This review will outline current knowledge regarding the pathogenesis of PFAPA, as well as treatment strategies and our clinical experience.

Keywords: PFAPA; Periodic fever; Recurrent fever.

Publication types

Review