Síndrome PFAPA: Diagnóstico e Tratamento na Atenção Primária à Saúde
DOI:
https://doi.org/10.5712/rbmfc14(41)1815Palavras-chave:
Febre Recorrente, Faringite, Estomatite Aftosa, Adenite, Atenção Primária à SaúdeResumo
Objetivo: Apresentar informações sobre o diagnóstico e tratamento da síndrome PFAPA na Atenção Primária à Saúde. Métodos: Revisão sistemática de literatura baseada na recomendação PRISMA e realizada nas bases de dados Scielo, Lilacs, Medline, IBECS e PubMed, incluindo estudos publicados no período de 2004 a 2018, além da consulta a outros documentos específicos da síndrome PFAPA. Resultados: Após busca e seleção, foram incluídos 31 artigos. Avaliação e Diagnóstico: A síndrome PFAPA acomete principalmente crianças, sendo caracterizada por febre periódica acompanhada por faringite, estomatite aftosa e/ou adenite cervical. Seu diagnóstico é clínico e por exclusão, baseado em critérios estabelecidos. Recomendações: Os episódios costumam responder a prednisona e, em graus variáveis, a cimetidina e colchicina. Casos refratários e acompanhados de hipertrofia tonsilar são candidatos a tonsilectomia, devendo ser encaminhados à avaliação otorrinolaringológica.
Downloads
Métricas
Referências
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
Downloads
Publicado
Como Citar
Edição
Seção
Licença
Ao submeterem um manuscrito à RBMFC, os autores mantêm a titularidade dos direitos autorais sobre o artigo, e autorizam a RBMFC a publicar esse manuscrito sob a licença Creative Commons Atribuição 4.0 e identificar-se como veículo de sua publicação original.
