Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome

J Pediatr. 2011 Dec;159(6):958-64. doi: 10.1016/j.jpeds.2011.06.004. Epub 2011 Jul 27.


Objective: To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.

Study design: 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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child, Preschool
  • Female
  • Fever*
  • Follow-Up Studies
  • Humans
  • Infant
  • Lymphadenitis* / diagnosis
  • Lymphadenitis* / therapy
  • Male
  • Neck
  • Pharyngitis* / diagnosis
  • Pharyngitis* / therapy
  • Stomatitis, Aphthous* / diagnosis
  • Stomatitis, Aphthous* / therapy
  • Syndrome
  • Time Factors
  • Young Adult