Periodic Fever with Pharyngitis, Aphthous Stomatitis and Cervical Adenitis Syndrome: A Rare Cause of Fever in Adults

  • Diogo Paixão Marques Internal Medicine Department, Hospital de Egas Moniz, Lisbon, Portugal
  • Sara Rocha Internal Medicine Department, Hospital de Egas Moniz, Lisbon, Portugal
  • Marta Manso Internal Medicine Department, Hospital de Egas Moniz, Lisbon, Portugal
  • Raquel Domingos Internal Medicine Department, Hospital de Egas Moniz, Lisbon, Portugal
DOI: https://doi.org/10.12890/2019_001041

Keywords

Periodic fever with pharyngitis,, aphthous stomatitis and cervical adenitis, PFAPA syndrome, adults

Abstract

Periodic fever with pharyngitis, aphthous stomatitis and cervical adenitis (PFAPA syndrome) is a common cause of periodic fever in children and usually manifests as episodes of fever recurring with a clockwork periodicity. Although rare after adolescence, adult patients with PFAPA syndrome may present with a wider range of symptoms and may lack the clockwork periodicity of fever. A 24-year-old patient presented with a 4-year history of periodic fever with pharyngitis and cervical adenitis. She also complained of vomiting, fatigue and sporadically presented with aphthous stomatitis. During crises, laboratory evaluation showed a moderate elevation of inflammatory markers. Blood cultures and ANA titres were negative. Immunoglobulins and serum ferritin levels were normal. After other causes of periodic fever had been excluded, a diagnosis of PFAPA syndrome was made.

VIEW THE ENTIRE ARTICLE

References

  • Lachmann H. Periodic fever syndromes. Best Pract Res Clin Rheumatol 2017;31:596–609.
  • Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr 1999;135:15.

  • Rigante D, Vitale A, Natale MF, et al. A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome. Clin Rheumatol 2017;36:463.

  • Yoshihara T, Imamura T, Yokoi K, et al. Potential use of procalcitonin concentrations as a diagnostic marker of the PFAPA syndrome. Eur J Pediatr 2007;166:621.

  • Dale DC, Bolyard AA, Aprikyan A. Cyclic neutropenia. Semin Hematol 2002;39:89–94.

    • Published: 2019-03-08

    Issue: Vol 6 No 3 (view)

    Section: Articles

    How to cite:
    1.
    Marques DP, Rocha S, Manso M, Domingos R. Periodic Fever with Pharyngitis, Aphthous Stomatitis and Cervical Adenitis Syndrome: A Rare Cause of Fever in Adults. EJCRIM 2019;6 doi:10.12890/2019_001041.

    Most read articles by the same author(s)