Pediatr. praxi. 2013;14(4):221-224

Recurrent fever in children

MUDr.Petra Król, prof.MUDr.Pavla Doležalová, CSc.

Klinika dětského a dorostového lékařství, VFN a 1. LF UK, Praha

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

Published: August 29, 2013  Show citation