Caracterización clínica del Síndrome de PFAPA en una Unidad de Pediatría de Atención Primaria: presentación de una serie de casos

Abstract

The PFAPA syndrome (Periodic Fever, Adenopathy, Pharingitis, Afthae) is part of the differential diagnosis of periodic fever in children. Although there is a dysregulation of the immune system, its etiology is not completely clear. The diagnostic criteria are simple but non-specific, so we must consider a large group of autoinflammatory diseases in our differential diagnosis. A single dose of glucocorticoids given at the onset of an episode can dramatically abort fever attacks in a few hours. Nevertheless, there isn’t an effective treatment for the prevention of the episodes. We propose a review of this syndrome as a result of five cases in our health care center.