Síndrome PFAPA (febre periódica, aftas orais, faringite e adenite cervical) em crianças e adultos
PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome in children and adults
Leonardo Oliveira Mendonça; Gabriella Melo Fontes Silva Dias; Natália Cândido de Sousa; Renata Francesco; Fábio Fernandes Morato Castro; Myrthes Toledo Barros
Resumo
A síndrome PFAPA (febre periódica, aftas orais, faringite e adenite cervical) é a forma mais comum das doenças autoinflamatórias. Trata-se de uma doença rara, não genética, bimodal, usualmente com início dos sintomas antes dos 5 anos de vida, que também pode prolongar-se ou iniciar-se na vida adulta. A prevalência é variável em todo o mundo, e não há dados estatísticos da doença no Brasil. Diversos pontos da doença são discutíveis, como os critérios diagnósticos, as avaliações laboratoriais, a ausência de mutações genéticas específicas e a abordagem terapêutica e prognóstica. Este trabalho tem como objetivo rever, de forma narrativa e crítica, aspectos relacionados ao diagnóstico e tratamento da síndrome PFAPA em adultos e crianças. Foram feitas buscas nas redes de dados PubMed, Bireme e Cochrane, utilizando o acrônimo PFAPA sem auxílio de qualquer filtro. Quanto ao diagnóstico, os critérios pediátricos foram adaptados para os adultos, com análises variadas e divergentes. Há a necessidade pujante de desenvolver um consenso com diretrizes de diagnóstico de pacientes em populações bem caracterizadas para melhor diagnosticar a doença. Já em relação ao tratamento, a cirurgia com remoção das tonsilas é considerada curativa, com necessidade de se manter acompanhamento após o procedimento para confirmação da remissão da doença. As drogas poupadoras de corticoide ainda são incertas, e o uso de imunobiológicos deve ser reservado somente para pacientes refratários ao tratamento cirúrgico e com grande impacto na qualidade de vida.
Palavras-chave
Abstract
PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome is the most common form of autoinflammatory disease. It is a rare, non-genetic, bi-modal disease, usually with onset of symptoms before the age of 5 years, which may also be prolonged or start in adult life. The prevalence varies worldwide and there are no specific statistical data in Brazil. Several aspects of the disease are debatable, such as diagnostic criteria, laboratory evaluations, absence of specific genetic mutations, and therapeutic and prognostic approach. This paper aims to review in a narrative and critical manner aspects related to diagnosis and treatment of PFAPA syndrome in adults and children. PubMed, Bireme and Cochrane databases were searched using the acronym PFAPA without any filter. Regarding diagnosis, pediatric criteria were adapted for use in adults, with varied and divergent analyses. There is a strong need to develop consensus diagnostic guidelines for patients in well-characterized populations to better diagnose the disease. Regarding treatment, tonsillectomy is considered curative, requiring follow-up after the procedure to confirm remission of the disease. Corticosteroid-sparing agents remain uncertain, and the use of immunobiological agents should be reserved only for patients who are refractory to surgical treatment and experience a great impact on quality of life.
Keywords
References
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Submitted date:
02/09/2019
Accepted date:
02/28/2019
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