Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome: Current Literature Review with a Case Report

Mahmut Demirtaş; Ahmet Onur Odabaşı

doi:10.4274/meandros.2014

Olgu Sunumu

Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome: Current Literature Review with a Case Report

Yıl 2018, Cilt: 19 Sayı: 1, 71 - 74, 30.04.2018

Mahmut Demirtaş , Ahmet Onur Odabaşı

Öz

Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis syndrome (PFAPA) is a frequently seen, important medical condition, which must be kept in mind in periodic fever etiology. Although its etiology is not clearly understood, autoimmune etiology is suspected due to response to steroids. There is no specific test for the diagnosis of disease. Diagnosis is based on symptoms and physical examination. Although medical treatment is the first choice for the management of the disease, tonsillectomy take over in treatment options for termination of attacks and permanent results. We have presented a patient that was performed tonsillectomy in our clinic for frequent attacks despite recurrent medical therapy with diagnosis of PFAPA with review of current literature.

Anahtar Kelimeler

Periodic fever, tonsillectomy, PFAPA

Kaynakça

1. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr 1987; 110: 43-6.
2. Vigo G, Zulian F. Periodic fevers with aphthous stomatitis, pharyngitis, and adenitis (PFAPA). Autoimmun Rev 2012; 12: 52-5.
3. Thomas KT, Edwards KM. Periodic fever syndrome. Pediatr Infect Dis J 1999; 18: 68-9.
4. Kuppersmith RB, Schams ST, Rosenfeld RM. Extracts from The Cochrane Library: Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Otolaryngol Head Neck Surg 2010; 143: 473-5.
5. Hofer M, Pillet P, Cochard MM, Berg S, Krol P, Kone-Paut I, et al. International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients. Rheumatology (Oxford) 2014; 53: 1125-9.
6. Akelma AZ, Cizmeci MN, Kanburoglu MK, Mete E, Bozkaya D, Tufan N, et al. Is PFAPA syndrome really a sporadic disorder or is it genetic? Med Hypotheses 2013; 81: 279-81.
7. Mahamid M, Agbaria K, Mahamid A, Nseir W. Vitamin D linked to PFAPA syndrome. Int J Pediatr Otorhinolaryngol 2013; 77: 362-4.
8. Vigo G, Martini G, Zoppi S, Vittadello F, Zulian F. Tonsillectomy efficacy in children with PFAPA syndrome is comparable to the standard medical treatment: a long-term observational study. Clin Exp Rheumatol 2014; 32(4 Suppl 84): 156-9.
9. 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 2008; 10: 358-60.
10. Stojanov S, Lapidus S, Chitkara P, Feder H, Salazar JC, Fleisher TA, et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade. Proc Natl Acad Sci U S A 2011; 108: 7148-53.
11. Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr 2010; 99: 178-84.
12. Burton MJ, Pollard AJ, Ramsden JD. Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Cochrane Database Syst Rev 2010; 8: 1-4.
13. Renko M, Salo E, Putto-Laurila A, Saxen H, Malttila PS, Luotonen J, et al. A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. J Pediatr 2007; 151: 289-92.
14. Garavello W, Romagnoli M, Gaini RM. Effectiveness of adenotonsillectomy in PFAPA syndrome: a randomized study. J Pediatr 2009; 155: 250-3.
15. Peridis S, Pilgrim G, Koudoumnakis E, Athanasopoulos I, Houlakis M, Parpounas K. PFAPA syndrome in children: A meta-analysis on surgical versus medical treatment. Int J Pediatr Otorhinolaryngol 2010; 74: 1203-8.

Yıl 2018, Cilt: 19 Sayı: 1, 71 - 74, 30.04.2018

Mahmut Demirtaş , Ahmet Onur Odabaşı

Öz

Kaynakça

1. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr 1987; 110: 43-6.
2. Vigo G, Zulian F. Periodic fevers with aphthous stomatitis, pharyngitis, and adenitis (PFAPA). Autoimmun Rev 2012; 12: 52-5.
3. Thomas KT, Edwards KM. Periodic fever syndrome. Pediatr Infect Dis J 1999; 18: 68-9.
4. Kuppersmith RB, Schams ST, Rosenfeld RM. Extracts from The Cochrane Library: Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Otolaryngol Head Neck Surg 2010; 143: 473-5.
5. Hofer M, Pillet P, Cochard MM, Berg S, Krol P, Kone-Paut I, et al. International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients. Rheumatology (Oxford) 2014; 53: 1125-9.
6. Akelma AZ, Cizmeci MN, Kanburoglu MK, Mete E, Bozkaya D, Tufan N, et al. Is PFAPA syndrome really a sporadic disorder or is it genetic? Med Hypotheses 2013; 81: 279-81.
7. Mahamid M, Agbaria K, Mahamid A, Nseir W. Vitamin D linked to PFAPA syndrome. Int J Pediatr Otorhinolaryngol 2013; 77: 362-4.
8. Vigo G, Martini G, Zoppi S, Vittadello F, Zulian F. Tonsillectomy efficacy in children with PFAPA syndrome is comparable to the standard medical treatment: a long-term observational study. Clin Exp Rheumatol 2014; 32(4 Suppl 84): 156-9.
9. 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 2008; 10: 358-60.
10. Stojanov S, Lapidus S, Chitkara P, Feder H, Salazar JC, Fleisher TA, et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade. Proc Natl Acad Sci U S A 2011; 108: 7148-53.
11. Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr 2010; 99: 178-84.
12. Burton MJ, Pollard AJ, Ramsden JD. Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Cochrane Database Syst Rev 2010; 8: 1-4.
13. Renko M, Salo E, Putto-Laurila A, Saxen H, Malttila PS, Luotonen J, et al. A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. J Pediatr 2007; 151: 289-92.
14. Garavello W, Romagnoli M, Gaini RM. Effectiveness of adenotonsillectomy in PFAPA syndrome: a randomized study. J Pediatr 2009; 155: 250-3.
15. Peridis S, Pilgrim G, Koudoumnakis E, Athanasopoulos I, Houlakis M, Parpounas K. PFAPA syndrome in children: A meta-analysis on surgical versus medical treatment. Int J Pediatr Otorhinolaryngol 2010; 74: 1203-8.

Toplam 15 adet kaynakça vardır.

Ayrıntılar

Birincil Dil	İngilizce
Konular	Kulak Burun Boğaz
Bölüm	Olgu Sunumu
Yazarlar	Mahmut Demirtaş Ahmet Onur Odabaşı
Yayımlanma Tarihi	30 Nisan 2018
Yayımlandığı Sayı	Yıl 2018 Cilt: 19 Sayı: 1

Kaynak Göster

EndNote	Demirtaş M, Odabaşı AO (01 Nisan 2018) Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome: Current Literature Review with a Case Report. Meandros Medical And Dental Journal 19 1 71–74.