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PFAPA Syndrome

by Stephen E. Goldfinger, MD

PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome causes recurrent episodes of fever that last 3 to 6 days, mouth sores (stomatitis), a sore throat (pharyngitis), and swollen lymph glands (adenitis). It typically starts between ages 2 and 5 years.

PFAPA syndrome is a relatively common periodic fever in children. Although it probably does not have a genetic cause, PFAPA is usually grouped with hereditary fever syndromes. PFAPA syndrome typically starts between the ages of 2 and 5 years and tends to be more common among boys.

About once a month, children have a fever that lasts 3 to 6 days. The syndrome causes fatigue, chills, and occasionally abdominal pain and headache, as well as fever, sore throat, mouth ulcers, and swollen lymph glands. Children are healthy between episodes, and growth is normal. Children tend to outgrow the syndrome.

A doctor usually bases the diagnosis on symptoms and the pattern in which they occur. Blood tests may be done to measure substances that indicate inflammation (called markers).

No treatment is required, but children may be given corticosteroids to relieve symptoms. Cimetidine seems to cure the syndrome in some children, as may removing the tonsils (tonsillectomy).

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