Abstract
Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) is a periodic fever syndrome in children. The etiology is unknown, and the course is usually self-limited taking 3–5 years to resolve. Treatment options include watchful waiting, medical management in the form of episodic oral steroids (for each episode which are very effective) or H2 blockers (which are used prophylactically and resolve the syndrome in less than 25 % of patients), or surgical management in the form of tonsillectomy (which resolve the syndrome in greater than 90 % of patients).
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 1987;110:43–6.
Abramson JS, Givner LB, Thompson JN. Possible role of tonsillectomy and adenoidectomy in children with recurrent fever and tonsillopharyngitis. Pediatr Infect Dis J. 1989;8:119–20.
Feder HM, Bialecki CA. Periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Pediatr Infect Dis J. 1989;8(3):186–7.
Thomas KT, Feder HM, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr. 1999;135(1):15–21.
Wurster VM, Carlucci JG, Feder HM, Edwards KM. Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. J Pediatr. 2011;159:958–64.
Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr. 2010;99:178–84.
Caorsi R, Pelagatti MA, Federici S, Finetti M, Martini A, Gattorno M. Periodic fever, apthous stomatitis, pharyngitis, and adenitis syndrome. Curr Opin Rheumatol. 2010;22:579–84.
Long S. Syndrome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA)-what it isn’t. What is it? J Pediatr. 1999;135(1):1–5.
Licameli G, Lawton M, Kenna M, Dedeoglu F. Long-term surgical outcomes of Adenotonsillectomy for PFAPA syndrome. Arch Otolaryngol Head Neck Surg. 2010;138(10):902–6.
Stojanov S, Lapidus S, Chitkara P, et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade. Proc Natl Acad Sci U S A. 2011;108:7148–53.
Feder HM. Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Pediatr Infect Dis J. 1992;11:318–21.
Burton MJ, Pollard AJ, Ramsden JD, Chong LY, Venekamp RP. Tonsillectomy for periodic fever, aphthous stomatitis and cervical adenitis syndrome (PFAPA). Cochrane Database Syst Rev. 2014;9, CD008669.
Peridis S, Pilgrim G, Koudoumnakis E, Athanasopoulos I, Houlakis M, Parpounas K. PFAPA syndrome in children: a meta-analysis on surgical versus medical treatment. Int J Pediatr Otorhinolaryngol. 2010;74:1203–8.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Kavanagh, K.R., Feder, H.M. (2016). PFAPA: Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis. In: Valdez, T., Vallejo, J. (eds) Infectious Diseases in Pediatric Otolaryngology. Springer, Cham. https://doi.org/10.1007/978-3-319-21744-4_16
Download citation
DOI: https://doi.org/10.1007/978-3-319-21744-4_16
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-21743-7
Online ISBN: 978-3-319-21744-4
eBook Packages: MedicineMedicine (R0)