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PFAPA syndrome: a review on treatment and outcome

Federica Vanoni1, Katerina Theodoropoulou2, Michaël Hofer3

  • 1Pediatric Department of Southern Switzerland, Ospedale Regionale di Bellinzona e Valli, Bellinzona, 6500, Switzerland. federica.vanoni@eoc.ch.

Pediatric Rheumatology Online Journal
|June 29, 2016
PubMed

Insights

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is common in children. While treatments like corticosteroids and colchicine exist, some cases may persist into adulthood, requiring further investigation.

Area of Science:

  • Pediatrics
  • Immunology
  • Rheumatology

Background:

  • Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequent cause of periodic fever in pediatric populations.
  • Current treatments include corticosteroids for acute episodes and colchicine for prophylaxis, with varying efficacy.
  • Tonsillectomy is a potential intervention for specific cases.

Purpose of the Study:

  • To review the current understanding and management of PFAPA syndrome.
  • To highlight the potential for PFAPA syndrome to persist into adulthood.

Main Methods:

  • Review of existing literature on PFAPA syndrome.
  • Analysis of treatment outcomes and long-term prognosis.

Main Results:

  • Corticosteroids are effective for fever management during episodes.
  • Colchicine can be used for preventing febrile episodes.
  • PFAPA syndrome typically resolves in adolescence, but adult persistence is increasingly recognized.

Conclusions:

  • PFAPA syndrome management involves symptomatic treatment and prophylaxis.
  • The possibility of adult PFAPA syndrome warrants further research and clinical consideration.

Keywords:
ColchicineGlucocorticoidsPFAPA outcomePFAPA treatmentTonsillectomy

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