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DISTINGUISHING CLINICAL AND LABORATORY FEATURES OF PATIENTS WITH PFAPA SYNDROME, RECURRENT TONSILLOPHARNGITIS AND INFECTIOUS TONSILLITIS

Year 2019, Volume: 13 Issue: 2, 107 - 111, 26.03.2019
https://doi.org/10.12956/tchd.511240

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.

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.

PFAPA SENDROMU, TEKRARLAYAN TONSİLLOFARENJİT VE ENFEKSİYÖZ TONSİLLOFARENJİT AYRIMINDA KLİNİK VE LABORATUVAR ÖZELLİKLER

Year 2019, Volume: 13 Issue: 2, 107 - 111, 26.03.2019
https://doi.org/10.12956/tchd.511240

Abstract

Giriş: Herediter ateş
sendromlarından biri olarak sınıflandırılan
PFAPA
(Periodic fever, apthous stomatitis, pharyngitis and cervical adenitis)
sendromu, periyodik ateş, farenjit, servikal lenfadenit ve stomatit ile
seyretmektedir.
PFAPA sendromu ekartasyon tanısı olup, hastalığın bulguları çocukluk çağında sık
görülen bakteriyel tonsillit, viral üst solunum yolu enfeksiyonu ile
örtüşmektedir. H
astalığın ataklarına özgül laboratuvar belirteci
bulunmamaktadır. Bu çalışmanın amacı tekrarlayan ateş ve tonsillit atakları
nedeniyle PFAPA sendromu ön tanısı ile yönlendirilen hastaların izlemde
aldıkları tanıları ve PFAPA atağı ile bakteriyel ve viral tonsillit arasında
laboratuvar değerlerindeki farkları ortaya koymaktır.



Metod: Bu çalışmaya PFAPA
şüphesi ile yönlendirilen toplam 69 çocuk dahil edilmiştir. Bu hastalar en az 2
atak sırasında tek bir hekim tarafından değerlendirilerek, PFAPA sendromu
tanısı almıştır. Ateş atakları düzenli aralıklarla tekrarlamayan ve diğer
hastalık bulguları olan hastalar tekrarlayan tonsillofarenjit olarak
değerlendirilmiştir. PFAPA hastalarının laboratuvar değerleri (lökosit,
eritrosit sedimentasyon hızı, C reaktif protein ve prokalsitonin), Grup A beta
hemolitik streptokok geçiren 9 hasta ve viral tonsillit geçiren 15 hasta ile
karşılaştırılmıştır.



Sonuçlar: Tekrarlayan
tonsillofarenjit tanısı alan 23 hastadan ikisi mutasyon analizi ile ailevi
Akdeniz ateşi (AAA), biri gastroözafajiyel reflü tanısı almış; kalan 20
hastanın ise 15’inin takiplerinde ateşi hiç olmamış, beşinin ise tanı
kriterlerinde belirtildiği gibi düzenli aralıklarla tekrarlayan ateşleri
gözlenmemiştir. İzlemde PFAPA tanısı alan 46 hasta ile tekrarlayan tonsillit
tanısı alanlar karşılaştırıldığında, PFAPA tanısı alan hastaların ailelerinde
istatistiksel anlamlı olarak daha fazla tekrarlayan tonsillit hikayesi mevcuttu
(%60,9) (p=0.041) ve yakınmalarının başlangıç yaşı daha küçüktü (p=0.022). PFAPA
hastaları ile Grup A streptokok tonsilliti geçiren hastalar ile
karşılaştırıldığında prokalsitonin seviyelerinde anlamlı fark saptanmazken
(p=0.053), PFAPA atağında sedimentasyon daha yüksek, lökosit sayısı daha düşük
saptandı (p değerleri 0.021 ve <0,01).



Tartışma: Beş yaşın altında
tekrarlayan ateş ve farenjit nedeniyle başvuran hastalarda, atak özelliklerinin
ve atak aralıklarının değerlendirilmesi PFAPA tanısının doğru konulması
açısından önemlidir. Hastaların aile hikayesi, tekrarlayan farenjit ve ailevi
Akdeniz ateşi açısından mutlaka sorgulanmalıdır. Prokalsitonin seviyelerinde
PFAPA atağı ve bakteriyel tonsillit arasında farklılık saptanmamıştır.

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.
There are 14 citations in total.

Details

Primary Language Turkish
Subjects ​Internal Diseases
Journal Section ORIGINAL ARTICLES
Authors

Fehime Kara Eroğlu

Publication Date March 26, 2019
Submission Date January 10, 2019
Published in Issue Year 2019 Volume: 13 Issue: 2

Cite

Vancouver Kara Eroğlu F. PFAPA SENDROMU, TEKRARLAYAN TONSİLLOFARENJİT VE ENFEKSİYÖZ TONSİLLOFARENJİT AYRIMINDA KLİNİK VE LABORATUVAR ÖZELLİKLER. Türkiye Çocuk Hast Derg. 2019;13(2):107-11.


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