A clinical review of 105 patients with PFAPA (a periodic fever syndrome)

doi:10.1111/j.1651-2227.2009.01554.x

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Wiley InterScience

Acta Pædiatrica

Volume 99 Issue 2, Pages 178 - 184

Published Online: 4 Nov 2009

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REVIEW ARTICLE

A clinical review of 105 patients with PFAPA (a periodic fever syndrome)

HM Feder ^1,2 , JC Salazar ^2,3

1.Division of Pediatric Infectious Diseases, Departments of Family Medicine and Pediatrics, Connecticut Children's Medical Center, Hartford, CT, USA
2.University of Connecticut Health Center, Farmington, CT, USA
3.Division of Pediatric Infectious Diseases, Department of Pediatrics, Connecticut Children's Medical Center, Hartford, CT, USA

Correspondence to Henry M. Feder, Jr. M.D., Departments of Family Medicine and Pediatrics, University of Connecticut Health Center, Farmington, CT 06030, USA.
Tel: 860 679 2928 |
Fax: 860 679 1350 |
Email: feder@nso2.uchc.edu

KEYWORDS

aphthous • periodic fever • PFAPA

ABSTRACT

Aims: We describe the presentations and clinical outcomes of pediatric patients diagnosed with PFAPA (Periodic Fever, Aphthous lesions, Pharyngitis, and cervical Adenitis).

Materials and methods: The medical records of children with recurrent fever and referred between 1998 and 2007 to a tertiary pediatric care hospital were reviewed. Children who met clinical criteria for PFAPA were then asked to participate in a follow-up study.

Results: One hundred and five children met study criteria for PFAPA which included at least six episodes of periodic fever. Most (62%) were males, the mean age at onset of PFAPA was 39.6 months (80% were <5 years at onset), the mean duration of individual fever episodes was 4.1 days, and the mean interval between episodes was 29.8 days. Accompanying signs and symptoms included aphthous stomatitis (38%), pharyngitis (85%), cervical adenitis (62%), headache (44%), vomiting with fever spikes (27%) and mild abdominal pain (41%). A prodrome (usually fatigue) preceded the fever in 62% of patients. Parents noted that when their child with PFAPA had fever, other family members remained well. Laboratory tests in patients with PFAPA were nonspecific. Individual episodes of fever usually resolved with a single oral dose (∼1 mg/kg) of prednisilone. The interval between fever episodes shortened in 50% of patients who used prednisilone. PFAPA resolved spontaneously (mean length 33.2 months) in 211105 (20%) patients. PFAF'A episodes continued (mean length 23 months) at the end of this study in 661105 (63%) patients. Cimetidine therapy was associated with the resolution of the fevers in 7/26 (27%) patients; tonsillectomy was associated with the resolution of the fevers in 11/11 (100%) patients.

Conclusion: PFAPA can usually be defined by its clinical characteristics. Individual febrile episodes usually resolve dramatically with oral prednisilone. The cause of PFAPA is unknown and research is needed to define its etiology. The overall prognosis for children with PFAPA is excellent.

Received 10 July 2009; revised 9 September 2009; accepted 22 September 2009.

DIGITAL OBJECT IDENTIFIER (DOI)

10.1111/j.1651-2227.2009.01554.x About DOI