title: Periodic fever, apthous stomatitis, pharyngitis and adenitis syndrome.
abstract: OBJECTIVE
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: UO Pediatria II, G. Gaslini Institute and Department of Pediatrics, University of Genoa, Genoa, Italy.
authors.forename: Roberta
authors.initials: R
authors.lastname: Caorsi
authors.forename: Maria Antonietta
authors.initials: MA
authors.lastname: Pelagatti
authors.forename: Silvia
authors.initials: S
authors.lastname: Federici
authors.forename: Martina
authors.initials: M
authors.lastname: Finetti
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
authors.forename: Marco
authors.initials: M
authors.lastname: Gattorno
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005335:Fever of Unknown Origin / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008199:Lymphadenitis / Q000175:diagnosis* / Q000188:drug therapy; D010612:Pharyngitis / Q000175:diagnosis* / Q000188:drug therapy; D013281:Stomatitis, Aphthous / Q000175:diagnosis / Q000188:drug therapy; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones