PFAPA Syndrome in a Young Adult with a History of Tonsillectomy
Department of Internal Medicine and Medical Specialties, Policlinico Umberto I, University of Rome Sapienza, Rome, Italy. Internal Medicine
(Impact Factor: 0.9).
01/2011; 50(3):223-5. DOI: 10.2169/internalmedicine.50.4421
Since its clinical definition in 1987, the syndrome called, "periodic fever, aphtous stomatitis, pharyngitis and cervical adenitis" syndrome (PFAPA) has been considered peculiar to pediatric age. In the recent literature there are a few case reports of PFAPA in adults. We describe a case of a 21-year-old female affected by PFAPA who presented a history of tonsillectomy at the age of four. To our knowledge this is the fourth case described with a diagnosis of PFAPA in an adult with a history of tonsillectomy during childhood. Although the role of tonsillectomy in the treatment of PFAPA is still controversial, due to the lack of definitive data in literature, this case suggests that fever episodes may relapse several years after surgery.
Available from: Orso Maria Lucherini
- "This syndrome does not have a documented genetic basis, and spontaneous resolution of fever episodes is commonly observed a few years after symptom onset (Marshall et al., 1987, 1989; Thomas et al., 1999). Recent medical literature has included dozens of suspected cases in adults as well, suggesting that it should be taken into consideration also in adults (Cavuoto and Bonagura, 2008; Padeh et al., 2008; Colotto et al., 2011; Cantarini et al., 2012d,e; Cazzato et al., 2013). "
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ABSTRACT: PFAPA syndrome is characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Tonsillectomy and adenotonsillectomy are surgical treatment options for this periodic fever syndrome, the significance of which will be discussed.
Between 2004 and 2010 we collected data of 36 patients with the diagnosis of PFAPA syndrome. Data analysis was carried out on the basis of structured questionnairs, patients' files as well as a systematic evaluation of international literature up to April 2011.
The average age for the appearance of PFAPA episodes was 22 months and they recurred for an average duration of 3-5 days every 14-33 days. During a PFAPA attack, aphthous stomatitis was present in 70% of the patients, pharyngitis was present in 93% and cervical adenitis in 96%. The family history for recurrent fever was positive in 4 of the patients. In 85% cortikosteroids were the only effective medicative treatment with no further symptoms until the next attack. Surgery (tonsillectomy±adenoidectomy) aborted the PFAPA episodes in 10 of 16 patients, in 2 patients the frequency of episodes decreased, 3 patients had no noticeable change and 1 patient died as a result of postsurgical bleeding at another institution. After an average duration of illness of 4 years the PFAPA syndrome was in spontaneous remission in 8 patients.
Tonsillectomy is an effective treatment option for the PFAPA syndrome. Contrary to a general indication the decision should be personalized considering the benefit vs. the risk of operation with the advice of an ENT specialist.
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ABSTRACT: Periodic fever with aphthous pharyngitis and adenitis (PFAPA) syndrome, which usually occurs in early childhood, features periodic high fever, aphthous stomatitis, tonsillitis, pharyngitis and cervical adenitis. Recurrent tonsillitis during fever becomes an issue for the otolaryngologist. We think PFAPA syndrome is often misdiagnosed as chronic tonsillitis. Although antibiotics are usually prescribed for acute exacerbated chronic tonsillitis, antibiotics are ineffective in PFAPA syndrome, and steroids should be used instead. Differentiating PFAPA syndrome from chronic tonsillitis is thus a priority. In recent reports on tonsillectomy efficacy, surgery is an option for managing PFAPA syndrome. In our cases of a 4-year-old girl diagnosed clinically with PFAPA syndrome, tonsillectomy proved effective. Unlike chronic tonsillitis, PFAPA syndrome recurs regularly every 3-8 weeks, enabling tonsillectomy to be timed relatively easily. We review the literature and discuss clinical PFAPA syndrome features and management.
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