Abstract
Introduction: PFAPA (Periodic
fever, apthous stomatitis, pharyngitis and cervical adenitis) is characterized
by recurrent periodic fever, pharnygitis, stomatitis and classified under the
hereditary fever syndromes. Diagnosis is based on exclusion of diseases as
bacterial and viral pharngitis commonly seen during childhood. There is no
specific laboratory test during attacks. In this study, we recruited patients
with complaints of recurrent fever and pharngitis and evaluated their final
diagnosis. Also we aimed to compare the laboratory findings of PFAPA patients
with bacterial and viral tonsillits.
Methods: Sixty-nine patients
with recurrent fever and pharngitis were included in this study. All patients
were evaluated by the same physician. Patients who had regular fever attacks
and charactersitic physical findings were diagnosed as PFAPA syndrome. Others
who do not have regular fever were diagnosed as recurrrent tonsillopharngitis.
Leucocyte and acute phase reactants (erytrocyte sedimentation rate, C reactive
protein, procalcitonin) levels of patients with recurrent fever were compared
with patients with group A streptococcus tonsillitis (n=9) and viral
tonsillitis (n=15).
Results: On follow-up 46
patients were diagnosed as PFAPA syndrome and 23 patients were diagnosed as
recurrent tonsillopharngitis. Among patients with recurrent tonsillopharngitis,
two patients were diagnosed as familial Mediterrenean fever (FMF), one was
diagnosed as gastroesophageal reflux (GER) on follow-up. Remaining 20 patients
had neither fever on follow-up or had irregular fever. When we compared
patients with PFAPA syndrome and recurrent tonsillipharngitis, patients with
PFAPA syndrome had statistically more patients with familiy history of
recurrent tonsillitis (%60.9) (p=0.041) and were younger at disease onset (p=0.022).
The procalcitonin levels of PFAPA patients were indifferent compared to bacterial
tonsillitis patients (p=0.053).
Conclusion:
Patients who admitted for recurrent fever and tonsillitis before 5 years of age
should be followed for attack characteristics for correct diagnosis of PFAPA
syndrome. Family history should be questioned for recurrent tonsillitis and
familial mediterrenean fever. Procalcitonin levels were indifferent between
PFAPA patients and bacterial tonsillitis.
Keywords: PFAPA syndrome, recurrent tonsillopharngitis
References
- 1. Masters SL, Simon A, Aksentijevich I, Kastner DL (2009) Horror autoinflammaticus: the molecular pathophysiology of autoinflammatory disease (*). Annu Rev Immunol 27:621-68.
- 2. Marshall GS, Edwards KM, Lawton AR (1989) PFAPA syndrome. Pediatr Infect Dis J 8:658-9.
- 3. Theodoropoulou K, Vanoni F, Hofer M (2016) Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome: a Review of the Pathogenesis. Curr Rheumatol Rep 18:18. doi: 10.1007/s11926-016-0567-y.
- 4. Adrovic A, Sahin S, Barut K, Kasapcopur O (2018) Familial Mediterranean fever and periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: shared features and main differences. Rheumatol Int. doi: 10.1007/s00296-018-4105-2.
- 5. Gazi U, Agada ME, Ozkayalar H, Dalkan C, Sanlidag B, Safak MA, et al. (2018) Tonsillar antimicrobial peptide (AMP) expression profiles of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) patients. Int J Pediatr Otorhinolaryngol 110:100-4.
- 6. Tejesvi MV, Uhari M, Tapiainen T, Pirttila AM, Suokas M, Lantto U, et al. (2016) Tonsillar microbiota in children with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) syndrome. Eur J Clin Microbiol Infect Dis 35:963-70.
- 7. Tasher D, Somekh E, Dalal I (2006) PFAPA syndrome: new clinical aspects disclosed. Arch Dis Child 91:981-4.
- 8. Vanoni F, Theodoropoulou K, Hofer M (2016) PFAPA syndrome: a review on treatment and outcome. Pediatr Rheumatol Online J 14:38. doi: 10.1186/s12969-016-0101-9.
- 9. Vanoni F, Federici S, Anton J, Barron KS, Brogan P, De Benedetti F, et al. (2018) An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA). Pediatr Rheumatol Online J 16:27. doi: 10.1186/s12969-018-0246-9.
- 10. Pehlivan E, Adrovic A, Sahin S, Barut K, Kul Cinar O, Kasapcopur O (2018) PFAPA Syndrome in a Population with Endemic Familial Mediterranean Fever. J Pediatr 192:253-55.
- 11. Kraszewska-Glomba B, Szymanska-Toczek Z, Szenborn L (2016) Procalcitonin and C-reactive protein-based decision tree model for distinguishing PFAPA flares from acute infections. Bosn J Basic Med Sci 16:157-61.
- 12. Yazgan H, Keles E, Yazgan Z, Gebesce A, Demirdoven M (2012) C-reactive protein and procalcitonin during febril attacks in PFAPA syndrome. Int J Pediatr Otorhinolaryngol 76:1145-7.
- 13. Yoshihara T, Imamura T, Yokoi K, Shibata M, Kano G, Osone S, et al. (2007) Potential use of procalcitonin concentrations as a diagnostic marker of the PFAPA syndrome. Eur J Pediatr 166:621-2.
- 14. Vijayan AL, Vanimaya, Ravindran S, Saikant R, Lakshmi S, Kartik R, et al. (2017) Procalcitonin: a promising diagnostic marker for sepsis and antibiotic therapy. J Intensive Care 5:51.
