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PFAPA Syndrome

By Apostolos Kontzias, MD, Assistant Professor of Medicine and Director, Autoinflammatory Clinic, Cleveland Clinic Foundation

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 nodes (adenitis). It typically starts between ages 2 and 5 years.

PFAPA syndrome is a relatively common periodic fever syndrome 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 of PFAPA syndrome on symptoms and the pattern in which they occur. Blood tests may be done to measure substances that indicate inflammation (called markers).

Treatment

  • Sometimes corticosteroids, cimetidine, tonsillectomy, or a combination

No treatment for PFAPA syndrome is required, but children may be given corticosteroids to relieve symptoms. Cimetidine seems to cure the syndrome in some children. If other treatments do not help, doctors sometimes remove the tonsils (tonsillectomy), which usually relieves symptoms. Another drug called anakinra may help some children.

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