Çocuklarda Periyodik Ateş, Aftöz Stomatit, Farenjit ve Adenit (PFAPA) Sendromu: 82 Hastanın Retrospektif Olarak Değerlendirilmesi

Year 2020, , 224 - 229, 29.10.2020

Sevliya Öcal Demir

https://doi.org/10.21673/anadoluklin.698210

Abstract

Amaç: Periyodik ateş, aftöz stomatit, farenjit ve adenit (PFAPA) sendromu ani yükselen ateş, stomatit, farenjit ve adenit ile karakterize olup çocukluk çağında en yaygın görülen tekrarlayan ateş sendromudur. Bu çalışmada, bu hastalığa dair farkındalığı artırmak amacıyla PFAPA sendromu tanısı almış olan çocuklara dair klinik veriler değerlendirilmiştir.

Gereç ve Yöntemler: Bu retrospektif çalışma 1 Ekim 2017—30 Haziran 2019 döneminde Diyarbakır Çocuk Hastalıkları Hastanesi Çocuk Enfeksiyon Polikliniği’nde PFAPA tanısıyla izlenmiş olan 82 hastayı kapsamıştır. Hastaların demografik özellikleri, klinik ve laboratuvar bulguları, gördüğü tedaviler ve tedaviye yanıtları ile ilgili veriler tıbbi kayıtlardan elde edilmiştir.

Bulgular: Hastaların 52’si erkek, 30’u kızdı. Medyan şikayet başlangıç yaşı 18 (1–120) ay, medyan tanı yaşı 53 (12–172) aydı. Seksen iki hastanın hepsinde ateş ve farenjit, 56’sında lenfadenit, 48’inde kriptik tonsilit, 28’inde stomatit mevcuttu. Laboratuvar tetkiklerinde lökositoz (%46,38), nötrofili (%59,7) ve lenfopeni (%4,47) tespit edildi. C-reaktif protein hastaların %86,56’sında pozitif olup medyan değer 69,9 (6,19–279) mg/dl idi. Eritrosit sedimantasyon hızı medyan değeri 36 (7–101) ml/saat idi. Otuz dört (%41,46) hastada ateş nedeniyle en az bir kez hastaneye yatırılma hikayesi vardı. Elli sekiz (%71) hastanın birinci derece akrabalarında sık tonsilit atakları mevcuttu ve bunlardan 21’i (%25,6) tonsilektomi geçirmişti. Tüm hastalarda metilprednisolon tedavisine dramatik yanıt alınmıştı.

Tartışma ve Sonuç: PFAPA sendromunda erken tanı gereksiz tetkik, tedavi ve hastaneye yatışları önlemek için önemlidir. Beş yaşından küçük olan ve yineleyen ateş ve tonsilit atakları ile getirilen, özellikle de ailesinde sık tonsilit geçirme veya tonsilektomi hikayesi olan çocuklarda ayırıcı tanıda PFAPA sendromu göz önünde bulundurulmalıdır.

Keywords

çocuklar, PFAPA, periyodik ateş

Supporting Institution

yok

The Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) Syndrome in Children: A Retrospective Evaluation of 82 Patients

Abstract

Aim: The periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, characterized by sudden fever, stomatitis, pharyngitis, and adenitis, is the most common periodic fever syndrome in children. In this study, we aimed to increase awareness of the disease by evaluating clinical data of pediatric patients diagnosed with PFAPA syndrome.

Materials and Methods: The retrospective study included 82 patients who had been on follow-up after a diagnosis of PFAPA syndrome at the Pediatric Infectious Diseases Outpatient Clinic of the Diyarbakır Children’s Hospital between 1 October 2017 and 30 June 2019. Patient data on demographic characteristics, clinical and laboratory findings, treatments received, and response to treatment were obtained from the medical records.

Results: Of the patients, 52 were male and 30 female. The median age at onset of complaints was 18 (1–120) months while the median age at diagnosis was 53 (12–172) months. Of the 82 patients, all had fever and pharyngitis, 56 lymphadenitis, 48 cryptic tonsillitis, and 28 stomatitis. Laboratory tests revealed leukocytosis (46.38%), neutrophilia (59.7%), and lymphopenia (4.47%). C-reactive protein was positive in 86.56% of all patients, with a median value of 69.9 (6.19–279) mg/dl. The median erythrocyte sedimentation rate was 34 (7–101) ml/hour. Thirty-four (41.46%) patients had a history of at least one hospitalization for fever. Fifty-eight (71%) patients had first-degree relatives with a history of frequent attacks of tonsillitis, of whom 21 (25.6%) underwent tonsillectomy. All patients showed dramatic response to treatment with methylprednisolone.

Discussion and Conclusion: In PFAPA syndrome, early diagnosis is important for preventing unnecessary testing, treatment, and hospitalization. PFAPA syndrome should be considered in the differential diagnosis of children under 5 years of age who present with recurrent fever and tonsillitis episodes, particularly those who have relatives with a history of frequent tonsillitis or tonsillectomy.

Keywords

children, periodic fever, PFAPA

References

• 1. Marshall GS, Edwards KM, Lawton AR. PFAPA syndrome (letter). Pediatr Infect Dis J. 1989;8:658–9.
• 2. Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis and aphthous stomatitis. J Pediatr. 1987;110:43–6.
• 3. Stojanov S, Lapidus S, Chitkara P, Feder H, Salazar JC, Fleisher TA, ve ark. 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.
• 4. Manthiram K, Nesbitt E, Morgan T, Edwards KM. Family history in periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome. Pediatrics. 2016;138(3):e20154572.
• 5. 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.
• 6. Thomas KT, Feder HM Jr, Lawton AR, EdwardsKM. Periodic fever syndrome in children. J Pediatr. 1999;135:15–21.
• 7. Manthiram K, Lapidus S, Edwards K. Unraveling the pathogenesis of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis through genetic immunologic, and microbiologic discoveries: an update. Curr Opin Rheumatol. 2017;29:493–9.
• 8. Manthhiram K. Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA syndrome). Erişim: www.uptodate.com/contents/periodic-fever-with-aphthous-stomatitis-pharyngitis-and-adenitis-pfapa-syndrome (erişildi: 9.11.2019).
• 9. Lantto U, Kolvunen P, Taplainen T, Renko M. Long-term outcome of classic an incomplete PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) syndrome after tonsillectomy. J Pediatr. 2016;179:172–7.
• 10. Adachi M, Watanabe A, Nishiyama A, OyazataY, Kamioka I, Murase M, ve ark. Familial cases of periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. J Pediatr. 2011;158:155–9.
• 11. Sampaio IC, Rodrigo MJ, Monterio Margues JG. Two siblings with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome. Pediatr Infect Dis J. 2009;28:254–5.
• 12. Antön-Martin P, Movilla RO, Martin SG, Allende LM, Rubio MTC, Gonzalez MFL, ve ark. PFAPA syndrome in siblings. Is there a genetic background? Eur J Pediatr. 2011;170:1563–8.
• 13. Di Gioia SA, Bedoni N, von Scheven-Gete A, Vanoni F, Superti-Furga, Hofer M, ve ark. Analysis of the genetic basis of periodic fever with aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Sci Rep. 2015;5:10200.
• 14. Brown KL, Wekel P, Osla V, Sundqvist M, Savman K, Fasth A, ve ark. Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. BMC Pediatr. 2010;10:65.
• 15. Long SS. Syndrome of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA)—what it isn’t, what is it? J Pediatr. 1999;135:1–5.
• 16. Dytrych P, Krol P, Kotrova M, Kuzilkova D, Hubacek P, Krol L, ve ark. Polyclonal, newly derived T cells with low expression of inhibitory molecule PD-1 in tonsils define the phenotype of lymphocytes in children with periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Mol Immunol. 2015;65:139–47.
• 17. American Academy of Pediatrics. Group A streptococcal infections. In: Kimberlin DW, Brady MT, Jackson MA, Long SS (ed.), Red Book: 2018–2021 Report of the Committee on Infectious Diseases, 31. ed. Itasca, IL/ABD: American Academy of Pediatrics; 2018:748–62.
• 18. Toprak D, Demı̇rdaL T, Aşçı Z, Orhan S, Çetı̇nkaya Z, Demı̇rtürk N. Sağlıklı okul çocuklarında nazofarinkste A grubu beta hemolitik streptokok taşıyıcılığı. Düzce Tıp Fakültesi Derg. 2008;2:26–9.
• 19. Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (periodic fever syndrome). Acta Paediatr. 2010;99:178–84.
• 20. Aviel YB, Tatour S, Baruch RG, Brik R. Colchicine as a therapeutic option in periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Semin Arthritis Rheum. 2016;45:471–4.
• 21. Tasher D, Somekh E, Dalal I. PFAPA syndrome: new clinical aspects disclosed. Arch Dis Child. 2006;91:981–4.
• 22. Tasher D, Stein M, Dalal I, Somekh E. Colchicine prophylaxis for frequent periodic fever, aphthous stomatitis, pharyngitis and adenitis episodes. Acta Paediatr. 2008;97:1090–2.
• 23. Dusser P, Hentgen V, Neven B, Kone-Paut I. Is colchicine an effective treatment in periodic fever, aphthous stomatitis, pharyngitis cervical (PFAPA) syndrome? Joint Bone Spine. 2016;83:46.
• 24. Licameli G, Lawton M, Kenna M, Dedeoglu F. Long-term surgical outcomes of adenotonsillectomy for PFAPA syndrome. Arch Otolaryngol Head Neck Surg. 2012;138:902–6.