Periodic fever, aphthous stomatitis, pharyngitis and adenitis

Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome is a medical condition, typically occurring in young children, in which high fever occurs periodically at intervals of about 3-5 weeks, frequently accompanied by aphthous-like ulcers, pharyngitis and/or cervical adenitis (cervical lymphadenopathy). The syndrome was described in 1987 and named two years later.^{[1] [2][3]}

Signs and symptoms

The key symptoms of PFAPA are those in its name: periodic high

The cause of PFAPA is unknown.[4] It is frequently discussed together with other periodic fever syndromes.^[3]

Possible causes include primarily genetic factors or it may be due to an initial infection.

The condition appears to be the result of a disturbance of innate immunity.

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Diagnosis

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Treatment

PFAPA syndrome typically resolves spontaneously. Treatment options are used to lessen the severity of episodes.[6] These treatments are either medical or surgical:

One treatment often used is a dose of a

There has been some evidence for the use of medications to reduce the frequency of flare-ups, including colchicine and cimetidine.^[7]

Surgical removal of the tonsils appears to be beneficial compared to no surgery in symptom resolution and number of future episodes.^[6] The evidence to support surgery is; however, of moderate quality.^[6]

Children with PFAPA have an impaired quality of life, which may be treated via individual counseling.^[8]

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.^{[2] [9] [10]} However, PFAPA has been found in adults and may not spontaneously resolve.^{[11][non-primary source needed]} Children with PFAPA experience lower physical, emotional, and psychosocial functioning.^[8] Their performance in school is also substantially impacted.^[8]

References