Original ArticleLong-Term Follow-Up of Children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome
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.
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Contributed equally.