Pediatria pre prax 3/2009

Periodic fever syndromes – PFAPA syndrome

Periodic fever is defined as recurrences of fever that last from a few days to a few weeks, separated by symptom-free intervals of variable duration. This pattern of fever can be caused by recurrent infections or neoplastic disorders, but also by noninfectious inflammatory disorders. It is important to review the medical history carefully in patients with recurrent febrile attacks. Patients with periodic fever that persists for more than two years rarely have infections or malignant disorders. Hereditary syndromes with periodic fever with diverse etiology (hyperIgD syndrome, familial Mediterranean fever, the tumor necrosis factor (TNF) receptor–associated periodic syndrome, the Muckle-Wells syndrome and the familial cold autoinflammatory syndrome) consist of a group of autoinflammatory conditions. These should be differentiated from a specific nonhereditary form of periodic fever called as PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, cervical adenopathy syndrome).

Keywords: periodic fever, HIDS, PFAPA syndrome, diagnosis.