Síndrome PFAPA

Abstract

PFAPA syndrome is characterized by periodic fever, pharyngitis, cervical adenitis and aphthous stomatitis. The bouts of fever can last for days or even weeks. Between crises, patients remain asymptomatic for variable periods. It appears before the age of five and has limited duration (4-8 years). Its aetiopathogeny is unknown. Corticoids are the treatment of choice. Tonsillectomy has been proposed as a solution but remains controversial. We present the case of a 4-year-old girl with PFAPA syndrome who underwent tonsillectomy in January, 2008, and we review the literature.

PFAPA syndrome in children: A meta-analysis on surgical versus medical treatment

“…A comparison of treatment with steroids or surgery [4,[11][12][13][15][16][17]19,20,23,24] did not show any statistically significant difference, confirming the effectiveness of both therapies for the resolution of PFAPA syndrome (P = 0.83; OR, 0.90; 95% CI, 0.36-2.26). All analyses are summarized in Table 2.…”

“…A total of 50 were excluded because they did not meet the inclusion criteria. 14 studies published between 1999 and 2009 matched the selection criteria and therefore were included in the analysis [3,4,[11][12][13][14][15][16][17]19,20,[22][23][24]. All studies, except two [4,13] were retrospective in nature (Fig.…”

“…The use of cimetidine [4,14,16,17,19,22] is also ineffective (P = 0.02; OR, 0.15; 95% CI, 0.03-0.75). There is evidence that the use of steroids [4,[11][12][13][15][16][17]19,20,23,24] is effective in the resolution of symptoms (P < 0.00001; OR, 43.82; 95% CI, 10.68-179.69).…”

“…Similar results to the original analysis were obtained in the meta-analysis on papers reporting !8 patients [3,4,[13][14][15][16][17]19,20], meta-analysis of studies in or after 2006 [4,11,13,[15][16][17]20], and meta-analysis of high quality studies [4,12,14,16,17,19,20].…”

Úlceras orales: diagnóstico diferencial y tratamiento

“…Síndromes asociados a ulceración oral Síndrome PFAPA Acrónimo de las palabras en inglés periodic fever, aphtae, pharyngitis y cervical adenopathies (fiebre periódica, aftas, faringitis y adenopatías cervicales), es una de las causas de fiebre periódica en pediatría y se caracteriza por episodios febriles recurrentes (cada 3 a 6 semanas), acompañados en el 65-80% de los casos de faringitis, aftas bucales y adenopatías cervicales 22 Síndrome de Behç et Trastorno sistémico inflamatorio, multigenético, de etiología desconocida. Las características clínicas incluyen úlceras orales y genitales, inflamación ocular, lesiones en la piel, así como manifestaciones articulares, vasculares, neurológicas, pulmonares, gastrointestinales y genitourinarias 23 Síndrome de Sweet Trastorno caracterizado por fiebre, lesiones en piel de carácter eritematoso infiltrativo, leucocitosis con neutrofilia e infiltración densa de la dermis por neutrófilos maduros 24 El tratamiento a prescribir (que sería extrapolable en buena medida a otros tipos de úlceras, como las descritas con anterioridad) lo condicionan la gravedad y sintomatología dolorosa de la enfermedad, la frecuencia de los episodios con úlceras y la tolerancia del paciente a la medicación (tabla 2) 13 .…”