Pediatria pre prax 5/2013

Recurrent fever in children

Recurrent fever in children may pose a major diagnostic challenge to pediatricians. In the last decade, a new group of diseases from the category of autoinflammatory syndromes, whose main manifestation is periodic fever, has been included in the diagnostic algorithm. These conditions are characterized by impairment of innate immune mechanisms. Recurrent fever episodes are accompanied by local inflammatory processes. The majority of periodic fever syndromes are caused by a genetic disorder that induces excessive stimulation of proinflammatory mechanisms. Cryopyrin-associated periodic syndromes (CAPS) and tumor necrosis factor receptor-associated periodic syndrome (TRAPS) are characterized by autosomal dominant inheritance. Familial Mediterranean fever (FMF) and mevalonate kinase-associated periodic syndrome (MAPS) are distinguished by autosomal recessive inheritance. Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA), wherein the genetic background has not yet been determined, are outside this classification.

Keywords: periodic fever, innate immunity, inflammation.