Periodic fever syndrome in children.
OBJECTIVE
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).


METHODS
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.
School of Medicine, Vanderbilt University, Nashville, Tennessee, USA.
K T
KT
Thomas
H M
HM
Feder
A R
AR
Lawton
K M
KM
Edwards
D017668:Age of Onset; D002675:Child, Preschool; D010505:Familial Mediterranean Fever / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D005260:Female; D005334:Fever / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D005500:Follow-Up Studies; D006801:Humans; D008199:Lymphadenitis / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D008297:Male; D010612:Pharyngitis / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D013281:Stomatitis, Aphthous / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy; D013577:Syndrome