PFAPA syndrome - new clinical aspects revealed

Diana Tasher ^1*, Eli Somekh ¹ and Ilan Dalal ¹

¹ E. Wolfson Medical Center, Holon, Israel

^* To whom correspondence should be addressed. E-mail: dtasher{at}gmail.com.

Accepted 16 March 2006

Abstract

Background: The recently described PFAPA syndrome is characterized by periodic fever, aphthous stomatitis, pharyngitis and adenitis. However, there is currently relatively little data on the natural history of this syndrome.

Objective: To describe the presentation, clinical course, physician's awareness, therapeutic response and long-term follow-up of children with PFAPA syndrome. Methods: Children with PFAPA syndrome, referred over a 5- year period (from January 1999 to January 2004) were enrolled in the study. Data were gathered from medical records, parents' interviews, physical examination and telephone calls.

Results: A total of 54 cases of PFAPA syndrome were evaluated. Our patients had higher rate of abdominal pain (65%) and lower rate of aphthous stomatitis (39%) as compared to previous reports. We identified four different patterns of disease evolution including relatively common (n=14, 26%) and newly described course of alternating remissions and relapses. The remissions lasted 8.5 months in average (range: 4-36 months). Diagnosis was established by primary pediatricians in 30/54 (56%). However, a significant delay in diagnosis was apparent (mean: 15 months). Attacks were aborted by a much lower dose of prednisone or equivalent corticosteroid (mean: 0.6 mg/kg/day, range: 0.15-1.5 mg/kg/day) than reported previously. Tonsillectomy was successful in the prevention of recurrence of further episodes in all 6 cases that underwent the procedure.

Conclusions: We describe several new characteristics of PFAPA syndrome in children, contributing to our knowledge of this relatively unrecognized but troublesome syndrome. Early diagnosis and appropriate treatment can significantly improve the quality of life of both patients and families.

Keywords: PFAPA, corticosteroids treatment, periodic fever, pharyngitis

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