Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome: the debate on diagnosis and treatment continues

PFAPA syndrome is defined by recurrent fever flares accompanied by ORL symptoms and alternating with asymptomatic periods. It is mostly a benign disease with a major impact on the quality of life of the child and his/her relatives; the outcome is generally favorable with spontaneous remission after a few years. Beside symptomatic medications during fever flares, the most used treatment consists of a single dose of corticosteroids at flare onset to interrupt the attack; fever revolves usually within a few hours, but the following flares may recur with a higher frequency. Other medications were also reported in case series of PFAPA patients: colchicine to increase the intervals between the attacks, the IL-1 blocker anakinra to treat the flare or cimetidine that showed no convincing effects. More randomized controlled studies are needed to help the treating physician to choose the best therapy, and real-life long-term data to evaluate the long-term outcome of children with PFAPA syndrome.