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Review
. 2021 Jun 27;7(3):166-173.
doi: 10.1016/j.wjorl.2021.05.004. eCollection 2021 Jul.

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

Alicia Wang  1 , Kalpana Manthiram  2 , Fatma Dedeoglu  3   4 , Greg R Licameli  1   4
Affiliations
Review

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

Alicia Wang et al. World J Otorhinolaryngol Head Neck Surg. .

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.

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Conflict of interest statement

None.

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References

    1. Marshall G.S., Edwards K.M., Butler J., Lawton A.R. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 1987;110:43–46. - PubMed
    1. Hofer M., Pillet P., Cochard M.M. International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients. Rheumatology (Oxford) 2014;53:1125–1129. - PubMed
    1. Gattorno M., Hofer M., Federici S. Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis. 2019;78:1025–1032. - PubMed
    1. Król P., Böhm M., Sula V. PFAPA syndrome: clinical characteristics and treatment outcomes in a large single-centre cohort. Clin Exp Rheumatol. 2013;31:980–987. - PubMed
    1. Padeh S., Brezniak N., Zemer D. Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome. J Pediatr. 1999;135:98–101. - PubMed