Síndrome PFAPA: Diagnóstico y Tratamiento en la Atención Primaria a la Salud
DOI:
https://doi.org/10.5712/rbmfc14(41)1815Palabras clave:
Fiebre Recurrente, Faringitis, Estomatitis Aftosa, Linfadenitis, Atención Primaria de SaludResumen
Objetivo: Presentar informaciones sobre el diagnóstico y tratamiento del síndrome PFAPA en la Atención Primaria a la Salud. Métodos: Revisión sistemática de literatura basada en la recomendación PRISMA y realizada en las bases de datos Scielo, Lilacs, Medline, IBECS y PubMed, incluyendo estudios publicados en el período 2004 a 2018, además de la consulta a otros documentos específicos del síndrome PFAPA. Resultados: Después de la búsqueda y selección, se incluyeron 31 artículos. Evaluación y Diagnóstico: El síndrome PFAPA acomete principalmente niños, siendo caracterizada por fiebre periódica acompañada por faringitis, estomatitis aftosa y/o adenitis cervical. Su diagnóstico es clínico y por exclusión, basado en criterios establecidos. Recomendaciones: Los episodios suelen responder a la prednisona y, en grados variables, a la cimetidina y colchicina. Los casos refractarios y acompañados de hipertrofia tonsilar son candidatos a tonsilectomía, debiendo ser encaminados a la evaluación otorrinolaringológica.
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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
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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:
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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
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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-
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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
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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
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