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Childhood PFAPA Syndrome Cases in a University Hospital in Turkey: A 10-Year Analysis

Year 2023, Volume: 45 Issue: 3, 73 - 78, 30.09.2023

Mahmut Ekici , Cemile Ece Çağlar Şimşek

https://doi.org/10.7197/cmj.1351454

Abstract

Aim: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by recurrent fever attacks every 3-6 weeks lasting 3-6 days, associated with at least one of the three main symptoms. This study aimed to evaluate the demographic, clinical features, laboratory findings, and effectiveness of the applied treatment in patients with PFAPA syndrome.
Materials and Methods: A total of 73 patients diagnosed with PFAPA syndrome, aged between 0-18 years, who presented to Sivas Cumhuriyet University Hospital Pediatric Outpatient Clinic between 01.01.2012 and 31.12.2022, were included in this study. Demographic data such as age, gender, symptoms, laboratory findings, treatment and efficacy of the treatment were analysed.
Results: Out of the 73 patients, 34 (46.6%) were female and 39 (53.4%) were male. The mean and median age at diagnosis were 3.85±1.47 years and 3.30 (1.1-7.5) years, respectively. The most common presenting complaint was fever. Fever was present in 64 (87.6%) of the 73 patients. Pharyngitis was observed in 56 (76.7%) patients, cervical adenitis in 26 (35.6%), cryptic tonsillitis in 12 (16.5%), and aphthous stomatitis in 15 (20.6%). Leukopenia and neutropenia were not detected in patients at the time of diagnosis. Neutrophilia was observed in 59 (80.8%) patients, and leukocytosis in 64 (87.7%) patients. Prior to treatment, CRP levels were found to be higher than the reference value of 8 mg/L in all patients. Prior to treatment, ESR values were within normal range (lower than 20 mm/h) in 10 (13.7%) patients and higher than 20 mm/h in 63 (86.3%) patients. Tonsillectomy was performed in 10 (13.7%) of 73 patients.
Discussion and Conclusion: The possibility of PFAPA syndrome should be considered in the differential diagnosis of patients younger than 5 years of age who present with recurrent episodes of fever and tonsillitis and whose fever does not decrease despite antibiotic treatment. In this way, early diagnosis can be made, unnecessary antibiotic use can be avoided and thus unnecessary investigations, treatment and hospitalisations can be prevented.

Keywords

Periodic fever PFAPA children

References

  • 1. Lazea C, Damian L, Vulturar R, Lazar C. PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort. Int J Gen Med. 2022 Aug 29;15:6871-80.
  • 2. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 1987;110:43–46.
  • 3. Otar Yener G, Aktaş İ, Altıntaş Meşe C, Çakan M. Does having MEFV gene sequence variants affect the clinical course and colchicine response in children with PFAPA syndrome? Eur J Pediatr. 2022 Nov 15:1-7. 4. Cochard M, Clet J, Le L, et al. PFAPA syndrome is not a sporadic disease. Rheumatology. 2010;49:1984–87.
  • 5. Dammeyer KL, Schneider A, April MM, Kahn PJ. Synchronous disease onset and flares in siblings with PFAPA. Pediatr Rheumatol Online J. 2022 Oct 5;20(1):87.
  • 6. Rydenman K, Fjeld H, Hätting J, Berg S, Fasth A. Epidemiology and clinical features of PFAPA: a retrospective cohort study of 336 patients in western Sweden. Wekell P.Pediatr Rheumatol Online J. 2022 Sep 15;20(1):82.
  • 7. Kanık A, Eliaçık K, Kanık ET, Demirçelik Y, Demir BK. A comparative study for the clinical features in children with PFAPA syndrome who were diagnosed before and after the age of five. Int J Pediatr Otorhinolaryngol. 2022 Jul;158:111153.
  • 8. Banday AZ, Joshi V, Arora K,et al. Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries-A decade of experience from North India. Front Immunol. 2022 Sep 20;13:958473.
  • 9. Espin Diaz PC, Singh K, Kher P, et al. Periodic Fever in Children: Etiology and Diagnostic Challenges. Cureus. 2022 Jul 25;14(7):e27239.
  • 10. Kaynak D, Yildiz M, Sahin S, et al. NLRP3 gene variants and serum NLRP3 levels in periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. Clin Rheumatol. 2022 Sep 10. doi: 10.1007/s10067-022-06370-1. Online ahead of print.
  • 11. Okamoto CT, Chaves HL, Schmitz MJ. Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome in children: a brief literature review. Rev Paul Pediatr. 2022 Jun 10;40:e2021087.
  • 12. Førsvoll JA, Onymar K. C-reactive protein in the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Acta Paediatr. 2007. PMID: 17937691.
  • 13. Levinsky Y, Butbul Aviel Y, Ahmad SA, et al. PFAPA flares observed during COVID outbreak: can emotional stress trigger PFAPA attacks? A multicenter cohort study. Pediatr Rheumatol Online J. 2022 Jul 8;20(1):46.
  • 14. Sopeña B, Araújo O, Freire M, Barrera-López L, José HR. Efficacy of Canakinumab in a Patient With Adult-Onset Glucocorticoid-Resistant Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome. Mod Rheumatol Case Rep. 2022 May 27:rxac043.
  • 15. Wang A, Manthiram K, Dedeoglu F, Licameli GR. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review. World J Otorhinolaryngol Head Neck Surg 2021 Jun 27;7(3):166-73.
  • 16. Yıldız M, Haslak F, Adrovic A, Ülkersoy İ, Gücüyener N, et all. Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: A Single-Center Experience. Turk Arch Pediatr 2022 Jan;57(1):46-52.doi: 10.5152/TurkArchPediatr.2021.21229.
  • 17. Soriano A, Soriano M, Espinosa G, et al. Autoinflammatory Diseases and PFAPA Syndrome: Evidence-Based Approach and Proposal of a Practical Guide. Front Immunol 2020 Jun 3;11:865 doi: 10.3389/fimmu.2020.00865. eCollection 2020.
  • 18. Wekell P, Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome PFAPA syndrome. Presse Med 2019 Feb;48(1 Pt 2):e77-e87.doi:10.1016/j.lpm.2018.08.016. Epub 2019 Jan 22.
  • 19. Rydenman K, Berg S, Karlsson-Bengtsson A, Fasth A, Wekell P. PFAPA syndrome - An important differential diagnosis in children with recurrent fever. Lakartidningen 2019 Oct 29;116:FP9U.
  • 20. Gaggiano C, Rigante D, Sota J, Grosso S, Cantarini L. Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review. Clin Rheumatol 2019 Jan;38(1):11-17.doi: 10.1007/s10067-018-4361-2.
  • 21. Batu ED, Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome: main features and an algorithm for clinical practice. Rheumatol Int 2019 Jun;39(6):957-970.doi: 10.1007/s00296-019-04257-0.
  • 22. Burton MJ, Pollard AJ, Ramsden JD, Chong LY, Venekamp RP. Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Cochrane Database Syst Rev 2019 Dec 30;12(12):CD008669.doi:10.1002/14651858.CD008669.pub3.

Türkiye’de Bir Üniversite Hastanesindeki Çocukluk Çağı PFAPA Sendromu Vakaları: 10 Yıllık Analiz

Year 2023, Volume: 45 Issue: 3, 73 - 78, 30.09.2023

Mahmut Ekici , Cemile Ece Çağlar Şimşek

https://doi.org/10.7197/cmj.1351454

Abstract

Amaç: Ateş, aftöz stomatit, farenjit ve servikal adenit (PFAPA) sendromu , üç ana belirtiden en az biriyle ilişkili, her 3-6 haftada bir tekrarlayan, 3-6 gün süren ateş atakları ile karakterizedir. Bu çalışmada, PFAPA sendromu hastalığının demografik, klinik özellikleri,laboratuvar bulguları ve uygulanan tedavinin etkinliğinin değerlendirilmesi amaçlanmıştır.
Gereç ve Yöntemler: Bu çalışmaya Sivas Cumhuriyet Üniversitesi Hastahanesi Pediatri Polikliniğine 01.01.2012-31.12.2022 tarihleri arasında başvuran 0-18 yaş grubu hastalardan PFAPA Sendromu tanısı alan 73 hasta dahil edildi. Çalışmaya dahil edilecek hastalarda yaş, cinsiyet gibi demografik verileri, semptomlar, laboratuvar bulguları, uygulanan tedavi ve tedavinin etkinliği incelendi.
Bulgular: Yetmiş üç hastanın 34’ü (%46,6) kız , 39’u (%53,4) erkekti. Ortalama ve medyan tanı yaşı ise sırasıyla 3,85±1,47 yıl ve 3,30 (1,1–7,5) yıldı. En yaygın başvuru şikayeti ateş idi. 73 hastadan 64’ünde (%87,6) ateş şikayeti mevcuttu. Hastaların 56’sında (%76,7) farenjit, 26’sında (%35,6) servikal lenfadenit, 12’sinde (%16,5) kriptik tonsilit, 15’inde (%20,6) aftöz stomatit mevcuttu. Tanı anında hastalarda lökopeni ve nötropeni saptanmadı. Nötrofil yüksekliği 59 (%80,8) hastada ve lökositoz 64 (%87,7) hastada görüldü. Tedavi öncesi tüm hastaların CRP değeri referans değeri olan 8 mg/L’den yüksek bulundu. Tedavi öncesi ESH değeri hastaların 10 (% 13,7)’unda normal değerlerde (20 mm/h’den düşük), 63 (% 86,3) hastada ise 20 mm/h’den yüksekti. 73 hastadan 10’una (%13,7) tonsillektomi uygulandı.
Tartışma ve Sonuç: Tekrarlayan ateş ve tonsilit atakları ile getirilen, antibiyotik tedavisine rağmen ateşi düşmeyen 5 yaşından küçük hastalarda ayırıcı tanıda PFAPA sendromu olasılığı göz önünde bulundurulmalıdır. Bu sayede erken tanı konabilir, gereksiz antibiyotik kullanımından kaçınılabilir ve böylelikle gereksiz tetkik, tedavi ve hastaneye yatışların önüne geçilebilir.

Keywords

Periyodik ateş PFAPA çocuklar

References

  • 1. Lazea C, Damian L, Vulturar R, Lazar C. PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort. Int J Gen Med. 2022 Aug 29;15:6871-80.
  • 2. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 1987;110:43–46.
  • 3. Otar Yener G, Aktaş İ, Altıntaş Meşe C, Çakan M. Does having MEFV gene sequence variants affect the clinical course and colchicine response in children with PFAPA syndrome? Eur J Pediatr. 2022 Nov 15:1-7. 4. Cochard M, Clet J, Le L, et al. PFAPA syndrome is not a sporadic disease. Rheumatology. 2010;49:1984–87.
  • 5. Dammeyer KL, Schneider A, April MM, Kahn PJ. Synchronous disease onset and flares in siblings with PFAPA. Pediatr Rheumatol Online J. 2022 Oct 5;20(1):87.
  • 6. Rydenman K, Fjeld H, Hätting J, Berg S, Fasth A. Epidemiology and clinical features of PFAPA: a retrospective cohort study of 336 patients in western Sweden. Wekell P.Pediatr Rheumatol Online J. 2022 Sep 15;20(1):82.
  • 7. Kanık A, Eliaçık K, Kanık ET, Demirçelik Y, Demir BK. A comparative study for the clinical features in children with PFAPA syndrome who were diagnosed before and after the age of five. Int J Pediatr Otorhinolaryngol. 2022 Jul;158:111153.
  • 8. Banday AZ, Joshi V, Arora K,et al. Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries-A decade of experience from North India. Front Immunol. 2022 Sep 20;13:958473.
  • 9. Espin Diaz PC, Singh K, Kher P, et al. Periodic Fever in Children: Etiology and Diagnostic Challenges. Cureus. 2022 Jul 25;14(7):e27239.
  • 10. Kaynak D, Yildiz M, Sahin S, et al. NLRP3 gene variants and serum NLRP3 levels in periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. Clin Rheumatol. 2022 Sep 10. doi: 10.1007/s10067-022-06370-1. Online ahead of print.
  • 11. Okamoto CT, Chaves HL, Schmitz MJ. Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome in children: a brief literature review. Rev Paul Pediatr. 2022 Jun 10;40:e2021087.
  • 12. Førsvoll JA, Onymar K. C-reactive protein in the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Acta Paediatr. 2007. PMID: 17937691.
  • 13. Levinsky Y, Butbul Aviel Y, Ahmad SA, et al. PFAPA flares observed during COVID outbreak: can emotional stress trigger PFAPA attacks? A multicenter cohort study. Pediatr Rheumatol Online J. 2022 Jul 8;20(1):46.
  • 14. Sopeña B, Araújo O, Freire M, Barrera-López L, José HR. Efficacy of Canakinumab in a Patient With Adult-Onset Glucocorticoid-Resistant Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome. Mod Rheumatol Case Rep. 2022 May 27:rxac043.
  • 15. Wang A, Manthiram K, Dedeoglu F, Licameli GR. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review. World J Otorhinolaryngol Head Neck Surg 2021 Jun 27;7(3):166-73.
  • 16. Yıldız M, Haslak F, Adrovic A, Ülkersoy İ, Gücüyener N, et all. Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: A Single-Center Experience. Turk Arch Pediatr 2022 Jan;57(1):46-52.doi: 10.5152/TurkArchPediatr.2021.21229.
  • 17. Soriano A, Soriano M, Espinosa G, et al. Autoinflammatory Diseases and PFAPA Syndrome: Evidence-Based Approach and Proposal of a Practical Guide. Front Immunol 2020 Jun 3;11:865 doi: 10.3389/fimmu.2020.00865. eCollection 2020.
  • 18. Wekell P, Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome PFAPA syndrome. Presse Med 2019 Feb;48(1 Pt 2):e77-e87.doi:10.1016/j.lpm.2018.08.016. Epub 2019 Jan 22.
  • 19. Rydenman K, Berg S, Karlsson-Bengtsson A, Fasth A, Wekell P. PFAPA syndrome - An important differential diagnosis in children with recurrent fever. Lakartidningen 2019 Oct 29;116:FP9U.
  • 20. Gaggiano C, Rigante D, Sota J, Grosso S, Cantarini L. Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review. Clin Rheumatol 2019 Jan;38(1):11-17.doi: 10.1007/s10067-018-4361-2.
  • 21. Batu ED, Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome: main features and an algorithm for clinical practice. Rheumatol Int 2019 Jun;39(6):957-970.doi: 10.1007/s00296-019-04257-0.
  • 22. Burton MJ, Pollard AJ, Ramsden JD, Chong LY, Venekamp RP. Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA). Cochrane Database Syst Rev 2019 Dec 30;12(12):CD008669.doi:10.1002/14651858.CD008669.pub3.
There are 21 citations in total.

Details

Primary Language Turkish
Subjects Medical Education
Journal Section Medical Science Research Articles
Authors

Mahmut Ekici 0000-0001-7434-578X

Cemile Ece Çağlar Şimşek 0000-0002-4860-6613

Publication Date September 30, 2023
Acceptance Date September 22, 2023
Published in Issue Year 2023Volume: 45 Issue: 3

Cite

AMA Ekici M, Çağlar Şimşek CE. Türkiye’de Bir Üniversite Hastanesindeki Çocukluk Çağı PFAPA Sendromu Vakaları: 10 Yıllık Analiz. CMJ. September 2023;45(3):73-78. doi:10.7197/cmj.1351454
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