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HNO Nachrichten

, Volume 48, Issue 2, pp 26–30 | Cite as

Periodische Fieberschübe und Pharyngitis bei Kindern

Das PFAPA-Syndrom: Tonsillektomie als Therapieoption!

  • Richard de la Chaux
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Das PFAPA-Syndrom ist eine seltene Erkrankung, die schon im frühen Kindesalter auftritt. Das Vollbild ist gekennzeichnet durch periodische Fieberschübe, begleitet von einer aphthösen Stomatitis, Pharyngitis und zervikalen Lymphadenitis. Neben der medikamentösen Therapie vor allem mit Steroiden beschreiben einige Publikationen ein komplettes Sistieren der Fieberschübe und der Symptomatik nach Tonsillektomie.

Literatur

  1. 1.
    J Pediatr. 1987 Jan;110(1):43-6. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. Marshall GS, Edwards KM, Buttler J, Lawton AR.Google Scholar
  2. 2.
    Acta Paediatr. 2013 Feb;102(2):187-92. doi: 10.1111/apa.12069. Epub 2012 Nov 27. Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study. Forsvoll J1, Kristoffersen EK, Oymar K.CrossRefPubMedGoogle Scholar
  3. 3.
    J Pediatr. 1999 Jul;135(1):15-21. Periodic fever syndrome in children. Thomas KT1, Feder HM Jr, Lawton AR, Edwards KM.Google Scholar
  4. 4.
    J Pediatr. 1999 Jul;135(1):98-101. Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome. Padeh S1, Brezniak N, Zemer D, Pras E, Livneh A, Langevitz P, Migdal A, Pras M, Passwell JH.Google Scholar
  5. 5.
    Pediatrics. 2016 Sep;138(3). pii: e20154572. doi: 10.1542/peds.2015-4572. Family History in Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome. Manthiram K1, Nesbitt E1, Morgan T2, Edwards KM3.CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Rheumatology (Oxford). 2010 Oct;49(10):1984-7. doi: 10.1093/rheumatology/keq187. Epub 2010 Jul 2. PFAPA syndrome is not a sporadic disease. Cochard M1, Clet J, Le L, Pillet P, Onrubia X, Gueron T, Faouzi M, Hofer M.CrossRefPubMedGoogle Scholar
  7. 7.
    Sci Rep. 2015 May 19;5:10200. doi: 10.1038/srep10200. Analysis of the genetic basis of periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Di Gioia SA1, Bedoni N1, von Scheven-Gete A2, Vanoni F2, Superti-Furga A3, Hofer M4, Rivolta C1.Google Scholar
  8. 8.
    Mediators Inflamm. 2015;2015:293417. doi: 10.1155/2015/293417. Epub 2015 Mar 4. Clinical features and genetic background of the periodic Fever syndrome with aphthous stomatitis, pharyngitis, and adenitis: a single center longitudinal study of 81 patients. Perko D1, Debeljak M2, Toplak N3, Av.in T3.CrossRefGoogle Scholar
  9. 9.
    Acta Paediatr. 2016 Oct;105(10):1140-51. doi: 10.1111/apa.13531. Epub 2016 Aug 19. Review of autoinflammatory diseases, with a special focus on periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome. Wekell P1,2, Karlsson A3, Berg S4,5, Fasth A4,5.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Clin Exp Rheumatol. 2016 Sep-Oct;34(6 Suppl 102):77-81. Epub 2016 Oct 25. Demographic, clinical and therapeutic findings in a monocentric cohort of adult patients with suspected PFAPA syndrome. Vitale A1, Orlando I1, Lopalco G2, Emmi G3, Cattalini M4, Frediani B1, Galeazzi M1, Iannone F2, Rigante D5, Cantarini L6.Google Scholar
  11. 11.
    Clin Rheumatol. 2017 Feb;36(2):463-468. doi: 10.1007/s10067-016-3317-7. Epub 2016 Jun 1. A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome. Rigante D1, Vitale A2, Natale MF1, Lopalco G3, Andreozzi L1, Frediani B2, DeErrico F1, Iannone F3, Cantarini L4,5.CrossRefPubMedGoogle Scholar
  12. 12.
    Laryngorhinootologie. 2002 Sep;81(9):635-9. [PFAPA Syndrome: Current Standard of Knowledge and Relevance for the ENT Specialist]. [Article in German] Ridder GJ1, Fradis M, Berner R, Lohle EGoogle Scholar
  13. 13.
    Intern Med. 2011; 50(3):223-5. PFAPA syndrome in a young adult with a history of tonsillectomy. Colotto M, Maranghi M, Durante C, Rossetti M, Renzi A, Anatra MG.Google Scholar
  14. 14.
    Rheumatology (Oxford). 2014 Jun;53(6):1125-9. International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients. Hofer M, Pillet P, Cochard MM, Berg S, Krol P, Kone-Paut I, Rigante D, Hentgen V, Anton J, Brik R, Neven B, Touitou I, Kaiser D, Duquesne A, Wouters C, Gattorno M.Google Scholar
  15. 15.
    Rheumatol Int. 2017 Jun;37(6):883-889. doi: 10.1007/s00296-017-3688-3. Epub 2017 Mar 7. Physicianse perspectives on the diagnosis and management of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Manthiram K1, Li SC2, Hausmann JS3,4, Amarilyo G5,6, Barron K7, Kim H8, Nativ S9, Lionetti G10, Zeft A11, Goldsmith D12, Kimberlin D13,14, Edwards K1, Dedeoglu F3, Lapidus S15; Childhood Arthritis and Rheumatology Research Alliance (CARRA) PFAPA Subcommittee.CrossRefPubMedGoogle Scholar
  16. 16.
    Mediators Inflamm. 2015;2015:563876. doi: 10.1155/2015/563876. Epub 2015 Sep 17. The Pathogenesis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Review of Current Research. Kraszewska-G.omba B1, Matkowska-Kocjan A1, Szenborn L1.CrossRefGoogle Scholar
  17. 17.
    Blood 2000; 96: 2317-22. Mutations in the gene encoding neutrophil elastase in congenital and cyclic neutropenia. Dale DC, Person RE, Bolyard AA, Aprikyan AG, Bos C, Bonilla MA et al.Google Scholar
  18. 18.
    Int J Pediatr Otorhinolaryngol. 2012 Nov;76(11):1588-90. doi: 10.1016/j.ijporl.2012.07.020. Epub 2012 Jul 31. Comparison of conventional and low dose steroid in the treatment of PFAPA syndrome: preliminary study. Yazgan H1, Gultekin E, Yaz.c.lar O, Sagun OF, Uzun L.CrossRefPubMedGoogle Scholar
  19. 19.
    Arch Dis Child. 2006 Dec;91(12):981-4. Epub 2006 Apr 4. PFAPA syndrome: new clinical aspects disclosed. Tasher D1, Somekh E, Dalal I.Google Scholar
  20. 20.
    Pediatr Int. 2017 Jun;59(6):655-660. doi: 10.1111/ped.13265. Epub 2017 Apr 24. Is colchicine more effective to prevent periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis episodes in Mediterranean fever gene variants? Gunes M1, Cekic S1, Kilic SS1.CrossRefPubMedGoogle Scholar
  21. 21.
    Semin Arthritis Rheum. 2016 Feb;45(4):471-4. doi: 10.1016/j.semarthrit.2015.07.005. Epub 2015 Jul 21. Colchicine as a therapeutic option in periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome. Butbul Aviel Y1, Tatour S2, Gershoni Baruch R3, Brik R4.CrossRefPubMedGoogle Scholar
  22. 22.
    Acta Paediatr. 2008 Aug;97(8):1090-2. doi: 10.1111/j.1651-2227.2008.00837.x. Epub 2008 May 7. Colchicine prophylaxis for frequent periodic fever, aphthous stomatitis, pharyngitis and adenitis episodes. Tasher D1, Stein M, Dalal I, Somekh E.CrossRefGoogle Scholar
  23. 23.
    Int J Pediatr Otorhinolaryngol. 2014 Jun;78(6):964-8. doi: 10.1016/j.ijporl.2014.03.026. Epub 2014 Apr 1. Vitamin D levels and effects of vitamin D replacement in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Stagi S1, Bertini F2, Rigante D3, Falcini F2.CrossRefPubMedGoogle Scholar
  24. 24.
    Pediatr Infect Dis. 1992; 11(4): 318-21. Cimetidine treatment for periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis. Feder HM.Google Scholar
  25. 25.
    Pediatr Infect Dis J. 1989 Feb;8(2):119-20. Possible role of tonsillectomy and adenoidectomy in children with recurrent fever and tonsillopharyngitis. Abramson JS1, Givner LB, Thompson JN.Google Scholar
  26. 26.
    J Pediatr. 2011 Jul;159(1):138-42. doi: 10.1016/j.jpeds.2010.12.014. Epub 2011 Feb 6. Tonsillectomy in children with periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome. Garavello W1, Pignataro L, Gaini L, Torretta S, Somigliana E, Gaini R.CrossRefPubMedGoogle Scholar
  27. 27.
    Arch Otolaryngol Head Neck Surg. 2012 Oct;138(10):902-6. doi: 10.1001/2013.jamaoto.313. Long-term surgical outcomes of adenotonsillectomy for PFAPA syndrome. Licameli G1, Lawton M, Kenna M, Dedeoglu F.CrossRefGoogle Scholar
  28. 28.
    J Pediatr. 2016 Dec;179:172-177.e1. doi: 10.1016/j.jpeds.2016.08.097. Epub 2016 Sep 28. Long-Term Outcome of Classic and Incomplete PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) Syndrome after Tonsillectomy. Lantto U1, Koivunen P2, Tapiainen T3, Renko M3.CrossRefPubMedGoogle Scholar
  29. 29.
    GMS Curr Top Otorhinolaryngol Head Neck Surg. 2016 Dec 15;15:Doc09. doi: 10.3205/cto000136. eCollection 2016. Indications for tonsillectomy stratified by the level of evidence. Windfuhr JP1.Google Scholar
  30. 30.
    Pediatr Rheumatol Online J. 2016 Jun 27;14(1):38. doi: 10.1186/s12969-016-0101-9. PFAPA syndrome: a review on treatment and outcome. Vanoni F1, Theodoropoulou K2, Hofer M3.Google Scholar
  31. 31.
    Eur J Clin Microbiol Infect Dis. 2016 Jun;35(6):963-70. doi: 10.1007/s10096-016-2623-y. Epub 2016 Mar 30. Tonsillar microbiota in children with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) syndrome. Tejesvi MV1, Uhari M2,3, Tapiainen T2,3, Pirttila AM1, Suokas M1, Lantto U2,4, Koivunen P2,4, Renko M5,6.CrossRefGoogle Scholar
  32. 32.
    Proc Natl Acad Sci U S A. 2011 Apr 26;108(17):7148-53. doi: 10.1073/pnas.1103681108. Epub 2011 Apr 8. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade. Stojanov S, Lapidus S, Chitkara P, Feder H, Salazar JC, Fleisher TA, Brown MR, Edwards KM, Ward MM, Colbert RA, Sun HW, Wood GM, Barham BK, Jones A, Aksentijevich I, Goldbach-Mansky R, Athreya B, Barron KS, Kastner DL.CrossRefGoogle Scholar

Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2018

Authors and Affiliations

  1. 1.MünchenDeutschland

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