Periodic fever syndrome in children,☆☆

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Abstract

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° C (confidence interval 40.4° to 40.6°). 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. (J Pediatr 1999;135:15-21)

Section snippets

METHODS

Patients were included in this study if they were evaluated by 1 of the authors or if there was telephone consultation with either parents or physicians of a child with periodic fevers. A PFAPA Registry form requested demographic information, birth and family history, characteristics of the febrile pattern, and associated symptoms. Medical records on each child were reviewed for clinical and laboratory data.

To determine the status of the febrile episodes and associated findings in these

Initial PFAPA Registry Patients

A total of 176 PFAPA Registry forms were returned to the authors. Of this number, 94 patients were identified with well-documented, regularly recurring periodic febrile episodes; 77 patients were excluded because the registry information did not clearly document the periodicity of the fever with asymptomatic intervening intervals. Five patients were excluded because they had diagnoses of other causes for periodic fevers including cyclic neutropenia (3 patients), Behcet syndrome (1 patient), and

DISCUSSION

The earliest reports of periodic fevers were presented in the 1940s by Reimann7 and Reimann and deBarardini.8 Using the term periodic to mean episodes recurring at regular intervals, Reimann described a group of patients with periodic fever beginning in infancy, persisting for years to decades, having cycles of similar duration, and a generally benign course.9, 10, 11 In 1974 he described an otherwise healthy man with high fever of 5 days’ duration, recurring at intervals of 20 to 23 days for 8

Acknowledgements

We are grateful for the assistance received from the multiple referring physicians and parents of the patients described.

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