Periodic fever syndrome in children

J Pediatr. 1999 Jul;135(1):15-21. doi: 10.1016/s0022-3476(99)70321-5.


Objectives: To describe the presentation, clinical course, therapeutic response, and long-term follow-up of patients with a syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA).

Study design: Patients with PFAPA (n = 94) referred over a 10-year period completed a registry form and provided medical records. Follow-up telephone calls were made in late 1997 to determine the persistence of episodes and sequelae.

Results: PFAPA episodes lasted 4.8 days (95% confidence interval 4.5 to 5.1) and recurred every 28 days (confidence interval 26 to 30), with a maximal temperature of 40.5 degrees C (confidence interval 40. 4 degrees to 40.6 degrees ). Of the 83 children available for follow-up, 34 no longer had episodes. In the remainder the episodes did not differ in character but recurred less frequently over time. The affected children had no long-term sequelae. Glucocorticoids were highly effective in controlling symptoms. Tonsillectomy and cimetidine treatment were associated with remission in a small number of patients.

Conclusions: PFAPA is a not uncommon cause of periodic fever in children. In some children the syndrome resolves, whereas symptoms in others persist. Long-term sequelae do not develop. The syndrome is easily diagnosed when regularly recurring episodes of fever are associated with aphthous stomatitis, pharyngitis, or cervical adenitis.

MeSH terms

  • Age of Onset
  • Child, Preschool
  • Familial Mediterranean Fever* / diagnosis
  • Familial Mediterranean Fever* / physiopathology
  • Familial Mediterranean Fever* / therapy
  • Female
  • Fever* / diagnosis
  • Fever* / physiopathology
  • Fever* / therapy
  • Follow-Up Studies
  • Humans
  • Lymphadenitis* / diagnosis
  • Lymphadenitis* / physiopathology
  • Lymphadenitis* / therapy
  • Male
  • Pharyngitis* / diagnosis
  • Pharyngitis* / physiopathology
  • Pharyngitis* / therapy
  • Stomatitis, Aphthous* / diagnosis
  • Stomatitis, Aphthous* / physiopathology
  • Stomatitis, Aphthous* / therapy
  • Syndrome