Abstract
Abstract Periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the main cause of noninfectious recurrent fever syndrome in young children. The diagnosis is mainly based on the clinical presentation, the exclusion of other causes for the attacks, and the absence of symptoms outside the flares. The etiology is only partially understood, but PFAPA is considered an autoinflammatory disease with increased IL-1β secretion, suspected to be related to polygenic gain of function variants. A single dose of steroids at the onset of fever is able to interrupt the flare in the majority of the patients, and tonsillectomy is often followed by a remission of the disease. The prognosis is good with a spontaneous remission before adulthood in the vast majority of the patients, and the absence of long-term damage.
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