Periodic fever, apthous stomatitis, pharyngitis and adenitis syndrome

Roberta Caorsi, Maria Antonietta Pelagatti, Silvia Federici, Martina Finetti, Alberto Martini, Marco Gattorno

Research output: Contribution to journalArticlepeer-review


Purpose Of Review: Periodic fever, apthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is the most common cause of periodic fever of unknown origin in childhood. During the last years a number of studies on large series of patients have shed more light on the actual clinical characterization, long-term outcome and response to treatment. Current PFAPA criteria have low specificity since they are positive in a considerable proportion of patients with inherited periodic fevers. We report on the findings coming from the analysis of large cohorts of PFAPA patients and the possible implication for the differential diagnosis. An update on the efficacy of possible prophylactic treatments and tonsillectomy is given. Recent Findings: A diagnostic score developed in a large series of children identifies patients meeting PFAPA criteria and at higher risk to carry relevant mutations of genes associated with periodic fevers. Randomized studies on the efficacy of tonsillectomy give a more evidence-based justification to this possible therapeutic approach. Summary: The findings coming from the recent literature give new information to clinicians for the correct diagnostic approach to pediatric and adult patients presenting periodic fever of unknown origin and provide an updated overview on the therapeutic possibilities for patients presenting a persistence of fever attacks.

Original languageEnglish
Pages (from-to)579-584
Number of pages6
JournalCurrent Opinion in Rheumatology
Issue number5
Publication statusPublished - Sep 2010


  • autoinflammatory diseases
  • differential diagnosis tonsillectomy
  • periodic fever

ASJC Scopus subject areas

  • Rheumatology
  • Medicine(all)


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