title: Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.
abstract: OBJECTIVE
  To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.
  
  
  METHODS
  Patients enrolled in a PFAPA registry were contacted and surveyed.
  
  
  RESULTS
  Patients in the registry (n = 59) were surveyed with a follow-up time ranging from 12 to 21 years. Fifty patients had complete symptom resolution, with mean symptom duration of 6.3 years (95% CI, 5.4-7.3), and no sequelae developed. Nine patients continued to have persistent symptoms for a mean duration of 18.1 years (95% CI, 17.4-18.8). There were no differences in initial presentation between subjects with resolved PFAPA and subjects with persistent PFAPA. In subjects with persistent PFAPA, the mean duration of fever >38.3°C decreased from 3.6 days at onset to 1.8 days at follow-up (P = .01), and the mean symptom-free interval between episodes increased from 29 to 159 days (P < .005). Thirty-seven of 44 patients treated with corticosteroids reported prompt symptom resolution. Twelve patients underwent tonsillectomy or adenotonsillectomy; 9 of these patients experienced markedly reduced symptoms, and 6 patients had resolution of symptoms. Two subjects received other diagnoses.
  
  
  CONCLUSIONS
  In long-term follow-up, most patients with PFAPA experienced spontaneous symptom resolution without sequelae. Patients with persistent symptoms had episodes of shorter duration and reduced frequency.
authors.affiliation: Department of Pediatrics and Division of Pediatric Infectious Diseases, Vanderbilt University, Nashville, TN, USA.
authors.forename: Victoria M
authors.initials: VM
authors.lastname: Wurster
authors.forename: James G
authors.initials: JG
authors.lastname: Carlucci
authors.forename: Henry M
authors.initials: HM
authors.lastname: Feder
authors.forename: Kathryn M
authors.initials: KM
authors.lastname: Edwards
mesh_terms: D002675:Child, Preschool; D005260:Female; D005334:Fever; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008199:Lymphadenitis / Q000175:diagnosis / Q000628:therapy; D008297:Male; D009333:Neck; D010612:Pharyngitis / Q000175:diagnosis / Q000628:therapy; D013281:Stomatitis, Aphthous / Q000175:diagnosis / Q000628:therapy; D013577:Syndrome; D013997:Time Factors; D055815:Young Adult