Periodic fever, aphthous stomatitis, pharyngitis and adenitis

Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is a medical condition, typically starting in young children, in which high fever occurs periodically at intervals of about 3-5 weeks, frequently accompanied by aphthous ulcers, pharyngitis and/or adenitis. The syndrome was described in 1987 and named two years later.

Signs and symptoms

The key symptoms of PFAPA are those in its name: periodic high fever at intervals of about 3-5 weeks, as well as aphthous ulcers, pharyngitis and/or adenitis. In between episodes, and even during the episodes, the children appear healthy. At least 6 months of episodes. Recurrent negative throat cultures. Other causes excluded (EBV, CMV, FMF).

Cause

PFAPA is frequently discussed together with other periodic fever syndromes, but it is unknown whether the cause is primarily genetic or due to an initial infection.

Treatment

A possible treatment for PFAPA is a single dose of prednisone (1-2 mg per kg body mass) at the beginning of each fever episode. The single dose usually terminates the fever within several hours. However, in some children, prednisone causes the fever episodes to occur more frequently (and more regularly) then consider colchicine like in FMF. In one study, Adenotonsillectomy was found to completely resolve symptoms. Yet other studies had differing results, most being positive. Parents of children with PFAPA regularly report improvements after the children have had surgical treatment. Some children stop having episodes, others have a break in the cycle for a few months and then it comes back but the episodes are less intense. In the majority of cases children outgrow this syndrome

Prognosis

According to present research, PFAPA does not lead to other diseases and spontaneously resolves as the child gets older, with no long term physical effects.