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2009, Número 3

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Acta Med 2009; 7 (3)


Síndromes de fiebre recurrente y su relevancia en la medicina contemporánea

Rojo EA

Idioma: Español
Referencias bibliográficas: 58
Paginas: 150-157
Archivo PDF: 82.22 Kb.


RESUMEN

Los síndromes autoinflamatorios (SAI) o también conocidos como síndromes de fiebre recurrente familiar (SFRF), son una entidad rara y poco conocida, con alta relevancia clínica. Son caracterizados por fiebre e inflamación grave recurrente sin causa aparente y con remisión espontánea. Los episodios se asocian a elevación de reactantes de fase aguda. Su principal característica es que no son mediados por anticuerpos o linfocitos T. Se originan en defectos genéticos que derivan en trastornos de regulación de la inmunidad innata. Se han descrito seis SFRF, entre los cuales se incluyen el síndrome periódico asociado al receptor del TNFα (TRAPS), la fiebre mediterránea familiar (FMF), hiper IgD (HIDS), síndrome de Muckle-Wells (MW), síndrome inflamatorio familiar relacionado al frío (FACS) y la enfermedad inflamatoria sistémica de inicio neonatal (NOMID/CINCA). MWS, FACS y CINCA también son conocidos como síndromes asociados a criopirinas. De manera más reciente se han descrito otros síndromes, como la fiebre periódica acompañada de estomatitis aftosa, faringitis y adenitis (PFAPA), el síndrome de artritis piogénica estéril, pioderma gangrenoso y acné (PAPA) y el síndrome Blau.


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