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.
Thomas
K T
KT
School of Medicine, Vanderbilt University, Nashville, Tennessee, USA.
Feder
H M
HM
Lawton
A R
AR
Edwards
K M
KM
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