PFAPA Syndrome in Child ENT Area and Its Indication for Tonsillectomy

Česká verzia

Authors: R. Katra; P. Dytrych ; D. Groh; M. Jurovčík; Z. Kabelka
Authors place of work: Subkatedra dětské otorinolaryngologie IPVZ, Praha přednosta doc. MUDr. Z. Kabelka, Ph. D. ; Klinika ušní, nosní a krční 2. LF UK a FN Motol, Praha
Published in the journal: Otorinolaryngol Foniatr, 59, 2010, No. 4, pp. 197-201.
Category: Original Article

Summary

PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis, Marshall’s syndrome is one of the periodic febrile syndromes and is meant to be an auto-inflammatory idiopathic disease. Recurrent (periodic) febrile episodes along with aphtous stomatitis, pharyngitis or tonsillitis and cervical adenitis (lymphadenopathy) are typical clinical signs. Another associated symptoms as vomiting, abdominal pain, diarrhea, arthritis, conjuctivitis or rash may occur. Children with initial manifestation of disease are usually younger than 5 years of age. Between acute onsets of attack they are entirely free of any clinical symptoms. The origin of this syndrome is unknown. The immune dysregulation of the tonsillar lymphatic tissue has been suspected. Diagnosis is difficult to confirm and is made by exclusion. During acute episodes nonspecific inflammatory markers are often elevated in laboratory studies. Corticosteroids can have a significant effect on resolution of symptoms. There is no causal treatment so far, surgical intervention can be considered based on assumption that adeno-tonsillectomy eliminates possible source of infection and remission of disease may occur. PFAPA syndrome causes periodic febrile episodes in early childhood relatively often. Differential diagnosis should consider and include several recurrent fever syndromes and their causalities, based on clinical studies and laboratory investigations and detailed history as well. An early diagnosis, appropriate therapeutic management and also more timely surgical intervention (tonsillectomy) may prevent costly and unnecessary investigations and will protect the patients from irrational and frequent antibiotic treatment.

Key words:
PFAPA syndrome, periodic fever, tonsillectomy, corticosteroids.

Zdroje

1. Berlucchi, M., Meini, A., Plebani, A. et al.: Update on treatment of Marshall’s syndrome (PFAPA syndrome): report of five cases with review of the literature. Ann. Otol Rhinol Laryngol, 112, 2003, s. 365-369.

2. Cortis, E., De Benedetti, F., Insalaco, A. et al.: Abnormal production of tumor necrosis factor (TNF) - alpha and clinical efficacy of the TNF inhibitor etanercept in a patient with PAPA syndrome [corrected]. J. Pediatr., 145, 2004, s. 851-855.

3. Dahn, K. A., Glode, M. P., Chan, K. H.: Periodic fever and pharyngitis in young children: a new disease for the otolaryngologist? Arch. Otolaryngol Head Neck Surg., 126, 2000, s. 1146-1149.

4. Feder, H. M., Jr.: Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Pediatr. Infect Dis. J., 11, 1992, s. 318-321.

5. Femiano, F., Lanza, A., Buonaiuto, C. et al.: Oral aphthous-like lesions, PFAPA syndrome: a review. J. Oral Pathol Med., 37, 2008, s. 319-323.

6. Forsvoll, J. A., Oymar, K.: C-reactive protein in the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Acta Pediatr., 96, 2007, s. 1670-1673.

7. Garavello, W., Romagnoli, M., Gaini, R. M.: Effectiveness of adenotonsillectomy in PFAPA syndrome: a randomized study. J. Pediatr., 155, 2009, s. 250-253.

8. Gattorno, M., Caorsi, R., Meini, A. et al.: Differentiating PFAPA syndrome from monogenic periodic fevers. Pediatrics, 124, 2009, s. e721-728.

9 Gattorno, M., Sormani, M. P., D’osualdo, A. et al.: A diagnostic score for molecular analysis of hereditary autoinflammatory syndromes with periodic fever in children. Arthritis Rheum, 58, 2008, s. 1823-1832.

10. Hofer, M. F.: Cured by tonsillectomy: was it really a PFAPA syndrome? J. Pediatr., 153, 2008, s. 298.

11. Król, P., Katra, R., Doležalová, P.: Syndromy periodických horeček. Čes.-slov. Pediat., 64, 2009, s. 480-487.

12. Leong, S. C., Karkos, P. D., Apostolidou, M. T.: Is there a role for the otolaryngologist in PFAPA syndrome? A systematic review. Int. J. Pediatr. Otorhinolaryngol, 70, 2006, s. 1841-1845.

13. Licameli, G., Jeffrey, J., Luz, J. et al.: Effect of adenotonsillectomy in PFAPA syndrome. Arch. Otolaryngol Head Neck Surg., 134, 2008, s. 136-140.

14. Lierl, M.: Periodic fever syndromes: a diagnostic challenge for the allergist. Allergy, 62, 2007, s. 1349-1358.

15. Marshall, G. S., Edwards, K. M., Butler, J. et al.: Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J. Pediatr., 110, 1987, s. 43-46.

16. Marshall, G. S., Edwards, K. M., Lawton, A. R.: PFAPA syndrome. Pediatr. Infect. Dis. J., 8, 1989, s. 658-659.

17. Padeh, S.: Periodic fever syndromes. Pediatr. Clin. North Am., 52, 2005, s. 577-609, vii.

18. Padeh, S., Brezniak, N., Zemer, D. et al.: Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: Clinical characteristics and outcome. J. Pediatr., 135, 1999, s. 98-101.

19. Padeh, S., Stoffman, N., Berkun, Y.: Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults. Isr. Med. Assoc. J., 10, 2008, s. 358-360.

20. Pignataro, L., Torretta, S., Pietrogrande, M. C. et al.: Outcome of tonsillectomy in selected patients with PFAPA syndrome. Arch. Otolaryngol Head Neck Surg., 135, 2009, s. 548-553.

21. Reimann, H. A.: Periodic disease; a probable syndrome including periodic fever, benign paroxysmal peritonitis, cyclic neutropenia and intermittent arthralgia. J. Am. Med. Assoc., 136, 1948, s. 239-244.

22. Simon, A., Van Der Meer, J. W., Vesely, R. et al.: Approach to genetic analysis in the diagnosis of hereditary autoinflammatory syndromes. Rheumatology (Oxford), 45, 2006, s. 269-273.

23. Stojanov, S., Hoffmann, F., Kery, A. et al.: Cytokine profile in PFAPA syndrome suggests continuous inflammation and reduced anti-inflammatory response. Eur Cytokine Netw., 17, 2006, s. 90-97.

24. Tasher, D., Somekh, E., Dalal, I.: PFAPA syndrome: new clinical aspects disclosed. Arch. Dis. Child, 91, 2006, s. 981-984.

25. Tasher, D., Stein, M., Dalal, I. et al.: Colchicine prophylaxis for frequent periodic fever, aphthous stomatitis, pharyngitis and adenitis episodes. Acta Pediatr., 97, 2008, s. 1090-1092.

26. Thomas, K. T., Edwards, K. M.: Periodic fever syndrome. Pediatr. Infect. Dis. J., 18, 1999, s. 68-69.

27. Thomas, K. T., Feder, H. M., Jr., Lawton, A. R. et al.: Periodic fever syndrome in children. J. Pediatr., 135, 1999, s. 15-21.

28. Tunca, M., Ozdogan, H.: Molecular and genetic characteristics of hereditary autoinflammatory diseases. Curr Drug Targets Inflamm Allergy, 4, 2005, s. 77-80.