Elsevier

The Journal of Pediatrics

Volume 159, Issue 6, December 2011, Pages 958-964
The Journal of Pediatrics

Original Article
Long-Term Follow-Up of Children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome

https://doi.org/10.1016/j.jpeds.2011.06.004Get rights and content

Objective

To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.

Study design

Patients enrolled in a PFAPA registry were contacted and surveyed.

Results

Patients in the registry (n = 59) were surveyed with a follow-up time ranging from 12 to 21 years. Fifty patients had complete symptom resolution, with mean symptom duration of 6.3 years (95% CI, 5.4-7.3), and no sequelae developed. Nine patients continued to have persistent symptoms for a mean duration of 18.1 years (95% CI, 17.4-18.8). There were no differences in initial presentation between subjects with resolved PFAPA and subjects with persistent PFAPA. In subjects with persistent PFAPA, the mean duration of fever >38.3°C decreased from 3.6 days at onset to 1.8 days at follow-up (P = .01), and the mean symptom-free interval between episodes increased from 29 to 159 days (P < .005). Thirty-seven of 44 patients treated with corticosteroids reported prompt symptom resolution. Twelve patients underwent tonsillectomy or adenotonsillectomy; 9 of these patients experienced markedly reduced symptoms, and 6 patients had resolution of symptoms. Two subjects received other diagnoses.

Conclusions

In long-term follow-up, most patients with PFAPA experienced spontaneous symptom resolution without sequelae. Patients with persistent symptoms had episodes of shorter duration and reduced frequency.

Section snippets

Methods

Patients with suspected PFAPA who were referred for clinical consultation to investigators at Vanderbilt University or the University of Connecticut from February 1988 to September 1997 were included in the original PFAPA registry. Inclusion in this registry was determined by adherence to these criteria: (1) regularly recurring fevers (>38.3°C), usually with an early age of onset; (2) constitutional symptoms in the absence of upper respiratory infection with at least one symptom being aphthous

Results

Between February 1988 and September 1997, the PFAPA registry grew to include 94 patients with well-documented PFAPA episodes. In 1999, a review of these 94 patients was published, including follow-up data for 83 of these patients. For this study, we attempted to contact the 83 patients who provided follow-up data from our original study. Of these 83 patients, 59 patients, their parents, or both were contacted, either via telephone, mail, or e-mail from February to October 2009 (Figure). All

Discussion

To our knowledge, this study is the largest series of patients with PFAPA reported with long-term follow-up.6, 7, 8, 9 Most other follow-up studies, although smaller in size, have shown results similar to ours, specifically that spontaneous resolution of PFAPA occurred commonly7, 9 and that corticosteroids and adenotonsillectomy were effective in modifying the symptoms of the disease. Further, our series and others have reported that no long-term sequelae or other co-morbidities developed.

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    Funding support from the Vanderbilt School of Medicine and Darlene Batey Hoffman Program. The authors declare no conflicts of interest.

    Contributed equally.

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