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A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome

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Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a mysterious disorder characterized by periodically recurrent fevers, oropharyngeal inflammation, and adenitis, which mainly affects children, though in very recent times, it has been also recognized in adulthood. We enrolled 115 unrelated pediatric and adult patients with history of periodic fevers who fulfilled the current diagnostic criteria for PFAPA syndrome in three Italian referral centers and highlighted differences between children and adults. Eighty-five children and 30 adults were evaluated: the frequency of flares was significantly higher in pediatric cases, while febrile attack duration was significantly longer in adults. Clockwork periodicity of fever and recurrent pharyngitis were more frequently observed in childhood, but no differences were identified for aphthosis and cervical adenopathy. Conversely, joint symptoms, myalgia, headache, fatigue, ocular signs, and rashes were more common in adults. The simultaneous occurrence of two or three cardinal PFAPA signs did not show any statistical difference between the groups, while the occurrence of only one cardinal manifestation was more frequent in adults. Corticosteroids were effective in 98.82 % of children and 88.2 % of adults. Tonsillectomy was rarely performed, resulting effective in only two patients. Our data illustrate the clinical overlap between pediatric and adult cases of PFAPA syndrome. Adults are characterized by a wider repertoire of inflammatory signs, suggesting that onset in adulthood might leave the disease misdiagnosed. Clinicians, not only pediatricians, should take into account this clinical entity in every patient of whatever age suffering from recurrent fevers of unknown origin.

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Acknowledgments

We thank all patients and/or their parents for the valuable help and cooperation in pursuing this study.

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Authors and Affiliations

  1. Institute of Pediatrics, Periodic Fever Research Center, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario “A. Gemelli”, Rome, Italy

    Donato Rigante, Marco Francesco Natale, Laura Andreozzi & Francesca D’Errico

  2. Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy

    Antonio Vitale, Bruno Frediani & Luca Cantarini

  3. Interdisciplinary Department of Medicine, Rheumatology Unit, University of Bari, Bari, Italy

    Giuseppe Lopalco & Florenzo Iannone

  4. Rheumatology Unit, Policlinico “Le Scotte”, University of Siena, viale Bracci 1, Siena, 53100, Italy

    Luca Cantarini

Authors
  1. Donato Rigante
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  2. Antonio Vitale
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  3. Marco Francesco Natale
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  4. Giuseppe Lopalco
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  5. Laura Andreozzi
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  6. Bruno Frediani
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  7. Francesca D’Errico
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  8. Florenzo Iannone
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  9. Luca Cantarini
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Corresponding author

Correspondence to Luca Cantarini.

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Patients’ medical records were reviewed for demographic and clinical data after obtaining informed consent from all families or individuals. The study was approved by the local ethical committee.

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Rigante, D., Vitale, A., Natale, M.F. et al. A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome. Clin Rheumatol 36, 463–468 (2017). https://doi.org/10.1007/s10067-016-3317-7

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  • DOI: https://doi.org/10.1007/s10067-016-3317-7

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