PFAPA Syndrome: Diagnosis and Treatment in Primary Health Care

Authors

DOI:

https://doi.org/10.5712/rbmfc14(41)1815

Keywords:

Relapsing Fever, Pharyngitis, Stomatitis, Aphthous, Lymphadenitis, Primary Health Care

Abstract

Objective: To present information about the diagnosis and treatment of PFAPA syndrome in Primary Health Care. Methods: Systematic review of literature based on the PRISMA recommendation and carried out in the Scielo, Lilacs, Medline, IBECS and PubMed databases, including studies published from 2004 to 2018, in addition to consulting other PFAPA syndrome specific documents. Results: After search and selection, 31 articles were included. Assessment and Diagnosis: PFAPA syndrome affects mainly children and is characterized by periodic fever accompanied by pharyngitis, aphthous stomatitis and/or cervical adenitis. Its diagnosis is clinical and by exclusion, based on established criteria. Recommendations: The episodes usually respond to prednisone and, in varying levels, cimetidine and colchicine. Refractory cases and accompanied by tonsillar hypertrophy are candidates for tonsillectomy, and should be referred to otorhinolaryngological evaluation.

Downloads

Download data is not yet available.

Metrics

Metrics Loading ...

Author Biographies

Laniel Aparecido Bueno, Universidade Estadual de Montes Claros (UNIMONTES), Montes Claros, Minas Gerais

Centro de Ciências Biológicas e da Saúde

Débora Magalhães Paiva, Universidade Estadual de Montes Claros (UNIMONTES), Montes Claros, Minas Gerais.

Centro de Ciências Biológicas e da Saúde.

Paulo Henrique Pimenta de Carvalho, Universidade Estadual de Montes Claros (UNIMONTES), Montes Claros, Minas Gerais.

Departamento de Clínica Cirúrgica, Centro de Ciências Biológicas e da Saúde

References

Marshall GS, Edwards KM, Lawton AR. PFAPA syndrome. Pediatr Infect Dis J. 1989;8(9):658-9. Disponível em: https://www.ncbi.nlm.nih.gov/pubmed/2797967 DOI: https://doi.org/10.1097/00006454-198909000-00026

Renko M, Salo E, Putto-Laurila A, Saxen H, Mattila PS, Luotonen J, et al. A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. J Pediatr. 2007;151(3):289-92. https://doi.org/10.1016/j.jpeds.2007.03.015

Long SS. Syndrome of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) – what It Isn’t. What Is It? J Pediatr. 1999;135(1):1-5. https://doi.org/10.1016/S0022-3476 (99)70316-1.

Colotto M, Maranghi M, Durante C, Rossetti M, Renzi A, Anatra MG. PFAPA syndrome in a young adult with a history of tonsillectomy. Intern Med. 2011;50(3):223-5. https://doi.org/10.2169/internalmedicine.50.4421 DOI: https://doi.org/10.2169/internalmedicine.50.4421

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(5):358-60. Disponível em: https://www.ima.org.il/MedicineIMAJ/viewarticle.aspx?year=2008&month=05&page=358

Fonseca AAR, Cherubini K. Aspectos de interesse clínico sobre a síndrome PFAPA (febre periódica, estomatite aftosa, faringite e adenite). Sci Med. 2005;15(1):68-73. Disponível em: http://revistaseletronicas.pucrs.br/ojs/index.php/scientiamedica/article/view/1546

Lantto U, Koivunen P, Tapiainen T, Renko M. Long-Term Outcome of Classic and Incomplete PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) Syndrome after Tonsillectomy. J Pediatr. 2016;179:172-7.e1. https://doi.org/10.1016/j.jpeds.2016.08.097 DOI: https://doi.org/10.1016/j.jpeds.2016.08.097

Moreira F, Pereira G, Marçal N, Guimarães J, Miranda D. Síndrome PFAPA - Caso clínico. Rev Port Otorrinolaringol Cir Cérvico-Facial. 2002;50(2):165-9. Disponível em: https://www.journalsporl.com/index.php/sporl/article/view/95

Silber M. Síndrome PFAPA (Periodic Fever, Adenitis, Pharyngitis, Aphthous Stomatitis) Conhecendo uma Doença Auto-inflamatória. In: Sih T, coord. VI Manual de Otorrinolaringologia Pediátrica da IAPO. Barueri: Gráfica e Editora RR Donnelley; 2007. p. 23-6. Disponível em: http://www.iapo.org.br/novo/secao.asp?s=47

Silber M. Síndrome PFAPA (Periodic Fever, Adenitis, Pharyngitis, Aphthous Stomatitis). In: Sih T, coord. IV Manual de Otorrinolaringologia Pediátrica da IAPO. Guarulhos: Lis Gráfica & Editora; 2005. p. 33-6. Disponível em: http://www.iapo.org.br/novo/secao.asp?s=45

Thomas KT, Feder HM Jr, Lawton AR, Edwards KM. Periodic fever syndrome in children. J Pediatr. 1999;135(1):15-21. https://doi.org/10.1016/S0022-3476(99)70321-5 DOI: https://doi.org/10.1016/S0022-3476(99)70321-5

Lawton A. Periodic fever with aphthous stomatitis, pharyngitis and adenitis (PFAPA syndrome). 2008. Disponível em: https://www.uptodate.com/pt/home

Tasher D, Somekh E, Dalal I. PFAPA syndrome: new clinical aspects disclosed. Arch Dis Child. 2006;91(12):981-4. https://doi.org/10.1136/adc.2005.084731 DOI: https://doi.org/10.1136/adc.2005.084731

De Cunto C, Britos M, Eymann A, Deltetto N, Liberatore D. Fiebre periódica: descripción de doce pacientes con fiebre periódica, estomatitis aftosa, faringitis y adenitis cervical. Arch Argent Pediatr. 2010;108(5):445-8. Disponível em:

http://www.scielo.org.ar/scielo.php?

script=sci_arttext&pid=S032500752010000500011&lng=es

Sampaio I, Marques JG. Síndrome PFAPA (Febre Periódica, Estomatite Aftosa, Faringite, Adenite) Análise Retrospectiva de 21 Casos. Acta Med Port. 2011;24(1):37-42. Disponível em: https://actamedicaportuguesa.com/revista/index.php/amp/article/download/345/115

Rigante D, Vitale A, Natale MF, Lopalco J, Andreozzi A, Frediani B, et al. A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome. Clin Rheumatol. 2016;36(2):463-8. https://doi.org/10.1007/s10067-016-3317-7 DOI: https://doi.org/10.1007/s10067-016-3317-7

Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatrica. 2010;99(2):178-84. https://doi.org/10.1111/j.1651-2227.2009.01554.x DOI: https://doi.org/10.1111/j.1651-2227.2009.01554.x

Król P, Böhm M, Sula V, Dytrych P, Katra R, Němcová D, et al. PFAPA syndrome: clinical characteristics and treatment outcomes in a large single-centre cohort. Clin Exp Rheumatol. 2013;31(6):980-7. Disponível em: http://www.clinexprheumatol.org/abstract.asp?a=6632

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(6):1125-9. https://doi.org/10.1093/rheumatology/ket460 DOI: https://doi.org/10.1093/rheumatology/ket460

Scattoni R, Verrotti A, Rinaldi VE, Paglino A, Carelli A, D’Alonzo R. Genital ulcer as a new clinical clue to PFAPA syndrome. Clin Exp Rheumatol. 2014;40(3):286-8. http://dx.doi.org/10.1111/ced.12524 DOI: https://doi.org/10.1111/ced.12524

Khodaghalian B, Tewary KK, Narchi H. PFAPA with facial swelling- a new association? Indian J Pediatr. 2013;80(5):425-7. https://doi.org/10.1007/s12098-012-0744-6 DOI: https://doi.org/10.1007/s12098-012-0744-6

Li CM, Wang CC, Lai CC, Fan HC, Huang WH, Cheng SN. Genital ulcers as an unusual sign of periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy syndrome: a novel Symptom? Pediatr Dermatol. 2011;28(3):290-4. https://doi.org/10.1111/j.1525-

2010.01247.x

Cantarini L, Vitale A, Bartolomei B, Galeazzi M, Rigante D. Diagnosis of PFAPA syndrome applied to a cohort of 17 adults with unexplained recurrent fevers. Clin Exp Rheumatol. 2012;30(2):269-71. Disponível em: http://www.clinexprheumatol.org/abstract.asp?a=5337

Førsvoll J, Kristoffersen EK, Øymar K. Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study. Acta Pædiatr. 2013;102(2):187-92. https://doi.org/10.1111/apa.12069 DOI: https://doi.org/10.1111/apa.12069

Shi T. Faringotonsilites Agudas. In: Sih T, coord. VI Manual de Otorrinolaringologia Pediátrica da IAPO. 6ª ed. Barueri: Gráfica e Editora RR Donnelley; 2007. p. 55-64. Disponível em: http://www.iapo.org.br/novo/secao.asp?s=47

Vanoni F, Theodoropoulou K, Hofer M. PFAPA syndrome: a review on treatment and outcome. Pediatr Rheumatol. 2016;14(1):38. https://doi.org/10.1186/s12969-016-0101-9 DOI: https://doi.org/10.1186/s12969-016-0101-9

Butbul Aviel Y, Tatour S, Gershoni Baruch R, Brik R. Colchicine as a therapeutic option in periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome. Semin Arthritis Rheum. 2016;45(4):471-4. https://doi.org/10.1016/j.semarthrit.2015.07.005 DOI: https://doi.org/10.1016/j.semarthrit.2015.07.005

Ciriza Barea E, Gonzalo Herrera NN, Galardi Andonegui MS, Leoz Oses P. Faringoamigdalitis de repetición sin causa infecciosa: síndrome PFAPA. A propósito de un caso. Acta Pediatr Esp. 2011;69(3):140-2. Disponível em: http://www.actapediatrica.com/index.php/secciones/notas-clinicas/36-faringoamigdalitis-de-repetici%C3%B3n-sin-causa-infecciosa-s%C3%ADndrome-pfapa-a-prop%C3%B3sito-de-un-caso#.W0TPl9VKjIU

Wurster VM, Carlucci JG, Feder HM Jr, Edwards KM. Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome. J Pediatr. 2011;159(6):958-64. https://doi.org/10.1016/j.jpeds.2011.06.004 DOI: https://doi.org/10.1016/j.jpeds.2011.06.004

Dusser P, Hentgen V, Neven B, Koné-Paut I. Is colchicine an effective treatment in periodic fever, aphtous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome? Joint Bone Spine. 2016;83(4):406-11. https://doi.org/10.1016/j.jbspin.2015.08.017 DOI: https://doi.org/10.1016/j.jbspin.2015.08.017

Tasher D, Stein M, Dalal I, Somekh E. Colchicine prophylaxis for frequent periodic fever, aphthous stomatitis, pharyngitis and adenitis episodes. Acta Paediatr. 2008;97(8):1090-2. https://doi.org/10.1111/j.1651-2227.2008.00837.x DOI: https://doi.org/10.1111/j.1651-2227.2008.00837.x

Stagi S, Bertini F, Rigante D, Falcini F. Vitamin D levels and effects of vitamin D replacement in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Int J Pediatr Otorhinolaryngol. 2014;78(6):964-8. https://doi.org/10.1016/j.ijporl.2014.03.026 DOI: https://doi.org/10.1016/j.ijporl.2014.03.026

Licameli G, Lawton M, Kenna M, Dedeoglu F. Long-term surgical outcomes of adenotonsillectomy for PFAPA syndrome. Arch Otolaryngol Head Neck Surg. 2012;138(10):902-6. https://doi.org/10.1001/2013.jamaoto.313 DOI: https://doi.org/10.1001/2013.jamaoto.313

Erdogan F, Kulak K, Öztürk O, İpek İÖ, Ceran Ö, Seven H. Surgery vs medical treatment in the management of PFAPA syndrome: a comparative trial. Paediatr Int Child Health. 2016;36(4):270-4. https://doi.org/10.1179/2046905515Y.0000000051 DOI: https://doi.org/10.1179/2046905515Y.0000000051

Garavello W, Pignataro L, Gaini L, Torretta S, Somigliana E, Gaini R. Tonsillectomy in children with periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome. J Pediatr. 2011;159(1):138-42. https://doi.org/10.1016/j.jpeds.2010.12.014 DOI: https://doi.org/10.1016/j.jpeds.2010.12.014

Pignataro L, Torretta S, Pietrogrande MC, Dellepiane RM, Pavesi P, Bossi A, et al. Outcome of tonsillectomy in selected patients with PFAPA syndrome. Arch Otolaryngol Head Neck Surg. 2009;135(6):548-53. https://doi.org/10.1001/archoto.2009.56 DOI: https://doi.org/10.1001/archoto.2009.56

Garavello W, Romagnoli M, Gaini RM. Effectiveness of adenotonsillectomy in PFAPA syndrome: a randomized study. J Pediatr. 2009;155(2):250-3. https://doi.org/10.1016/j.jpeds.2009.02.038 DOI: https://doi.org/10.1016/j.jpeds.2009.02.038

Licameli G, Jeffrey J, Luz J, Jones D, Kenna M. Effect of adenotonsillectomy in PFAPA syndrome. Arch Otolaryngol Head Neck Surg. 2008;134(2):136-40. https://doi.org/10.1001/archoto.2007.7 DOI: https://doi.org/10.1001/archoto.2007.7

Wong KK, Finlay JC, Moxham JP. Role of Tonsillectomy in PFAPA Syndrome. Arch Otolaryngol Head Neck Surg. 2008;134(1):16-9. https://doi.org/10.1001/archoto.2007.15 DOI: https://doi.org/10.1001/archoto.2007.15

Renko M, Salo E, Putto-Laurila A, Saxen H, Mattila PS, Luotonen J, et al. A randomized, controlled trial of tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome. J Pediatr. 2007;151(3):289-92. https://doi.org/10.1016/j.jpeds.2007.03.015 DOI: https://doi.org/10.1016/j.jpeds.2007.03.015

Pinto A, Lindemeyer RG, Sollecito TP. The PFAPA syndrome in oral medicine: differential diagnosis and treatment. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006;102(1):35-9. https://doi.org/10.1016/j.tripleo.2005.12.023 DOI: https://doi.org/10.1016/j.tripleo.2005.12.023

Published

2019-03-18

How to Cite

1.
Bueno LA, Paiva DM, Carvalho PHP de. PFAPA Syndrome: Diagnosis and Treatment in Primary Health Care. Rev Bras Med Fam Comunidade [Internet]. 2019 Mar. 18 [cited 2024 Jul. 22];14(41):1815. Available from: https://rbmfc.org.br/rbmfc/article/view/1815

Issue

Section

Clinical Reviews

Plaudit