PFAPA syndrome - Clinical case

Authors

  • Filipa Moreira Interna de Formação Específica, Hospital de Braga, Portugal
  • Gabriel Pereira Interno de Formação Específica, Hospital de Braga, Portugal
  • Nuno Marçal Interno de Formação Específica, Hospital de Braga, Portugal
  • Joana Guimarães Interna de Formação Específica, Hospital de Braga, Portugal
  • Daniel Miranda Interno de Formação Específica, Hospital de Braga, Portugal

DOI:

https://doi.org/10.34631/sporl.95

Keywords:

PFAPA syndrome, relapsing fever, corticosteroids, tonsillectomy

Abstract

Introduction: The PFAPA (periodic fever, aphtous stomatitis,
pharyngitis, cervical adenitis) syndrome is characterized
by recurrent episodes of high fever, aphtous stomatitis,
pharyngitis and cervical adenitis occurring mostly in children
younger than five years. The etiology is unknown and the
diagnosis is clinical and made by exclusion. This paper aims
to review literature - clinical, diagnosis and treatment -
complemented with the presentation of a clinical case.

Materials and methods: Literature search and document
analysis conducted through the database of Medline, Pubmed
and Ovid.

Results: The use of antibiotics or cimetidine is ineffective,
while corticosteroids and tonsillectomy, with or without
adenoidectomy, reduce the symptoms.

Conclusions: PFAPA syndrome is a rare disease, which
recognition can be difficult. The most effective medical
treatment are corticosteroids which, however, do not prevent
future recurrences. The most effective therapy in long-term
resolution of symptoms is tonsillectomy.

References

Marshall GS, Edwards KM, Lawton AR. PFAPA syndrome. Pediatr Infect Dis J 1989; 8:186-7.

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.

Fonseca AAR, Cherubini K. Clinical aspects of PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis and adenitis). Scientia Medica 2005; Jan./Mar.;15 (1): 68-73.

Padet S, Stoffman N, Berkun Y. Periodic Fever Accompanied by Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome (PFAPA Syndrome) in Adults. IMAJ 2008; 10: 358-360.

Licameli G, Jeffrey J, Luz J, Jones D e tal. Effect of Adenotonsillectomy in PFAPA Syndrome. Arch Otolaryngol Head Neck Surg. 2008; 134 (2):136-140.

Parich SR, Reiter ER, Kenna MA, Roberson D. Utility of Tonsillectomy in 2 Patients with the Syndrome of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis. Arch Otolaryngol Head Neck Surg. 2003; 129: 670-673.

Wong KK, Finlay JC, Moxham P. Role of Tonsillectomy in PFAPA Syndrome. Arch Otolaryngol Head Neck Surg. 2008; 134 (1):16-19.

Berlucchi M, Nicolai P. Marshall’s syndrome or PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome. Orphanet encyclopedia. Janeiro 2004; pp 1-5 www.orpha.net/data/phatho/GB/uk-PFAPA.pdf acedido a 22 Fevereiro 2011.

Galanakis E, Papadakis CE, Giannoussi E, Karatzanis AD et al. PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 2002; 86:434-435

Dahn KA, Glode MP, Chan KH. Periodic Fever and Pharyngitis in Young Children. Arch Otolaryngol Head Neck Surg. 2000; 126: 1146-1149.

Peridis S, Pilgrim G, Koudoumnakis E, Athanasopoulos I et al. PFAPA syndrome in children: A meta-analysis on surgical versus medical treatment. Int J Pediatr Otorhinolaryngol 2010; (74) 11: 1203-1208.

How to Cite

Moreira, F., Pereira, G., Marçal, N., Guimarães, J., & Miranda, D. (2012). PFAPA syndrome - Clinical case. Portuguese Journal of Otorhinolaryngology and Head and Neck Surgery, 50(2), 165–169. https://doi.org/10.34631/sporl.95

Issue

Section

Case Report