Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review

Carla Gaggiano, Donato Rigante, Jurgen Sota, Salvatore Grosso, Luca Cantarini

Research output: Contribution to journalArticlepeer-review

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequent non-hereditary autoinflammatory disorder in childhood: Its onset is usually observed before 5 years, though reports regarding adulthood are increasing. The pathogenesis of the syndrome is not completely understood, but a multifactorial origin, probably based on a polygenic pattern of susceptibility, is the most probable rational pathogenetic hypothesis. Treatment of PFAPA syndrome relies on the administration of low-dose corticosteroids, which promptly abort flares but cannot prevent subsequent disease episodes over time. Tonsillectomy with or without adenoidectomy has proved to be successful in some pediatric patients, as proven by different studies. On the other hand, colchicine, cimetidine, nonsteroidal anti-inflammatory drugs, and interleukin-1 inhibitors have shown efficacy, which require further definite confirmations. This review is aimed at summarizing all the recent evidence about treatment options available for PFAPA syndrome both in pediatric and adult patients.

Original languageEnglish
JournalClinical Rheumatology
DOIs
Publication statusAccepted/In press - Jan 1 2018

Keywords

  • Aphthous stomatitis
  • Autoinflammation
  • Cimetidine
  • Colchicine
  • Corticosteroids
  • Familial Mediterranean fever
  • Innovative biotechnologies
  • Interleukin-1
  • MEFV
  • Nonsteroidal anti-inflammatory drugs
  • Periodic fever
  • Personalized medicine
  • Pharyngitis and cervical adenitis (PFAPA) syndrome
  • Tonsillectomy

ASJC Scopus subject areas

  • Rheumatology

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