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PFAPA Syndrome

By

Gil Amarilyo

, MD, Tel Aviv University

Reviewed/Revised Dec 2023
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PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome is the most common periodic fever syndrome Overview of Hereditary Periodic Fever Syndromes Hereditary periodic fever syndromes are hereditary disorders that periodically cause episodes of fever and other symptoms that are not due to usual childhood infections or any other obvious... read more Overview of Hereditary Periodic Fever Syndromes 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. It has been recognized in adults as well.

About once a month, children have a fever that typically lasts 3 to 6 days. PFAPA syndrome causes fatigue, chills, and occasionally abdominal pain and headache, as well as fever, sore throat, mouth ulcers, and swollen lymph nodes.

Children are healthy between episodes, and growth is normal.

A doctor usually bases the diagnosis of PFAPA syndrome on symptoms and the pattern in which they occur. Blood tests (such as C-reactive protein and erythrocyte sedimentation rate, sometimes called inflammatory markers) may be done to measure inflammation.

Treatment of PFAPA Syndrome

  • Sometimes corticosteroids, colchicine, cimetidine, tonsillectomy, or a combination

No treatment for PFAPA syndrome is required, but children may be given corticosteroids to relieve symptoms. Cimetidine or colchicine seems to be helpful in some children. If these treatments do not help, doctors rarely remove the tonsils (tonsillectomy), which usually relieves symptoms.

Anakinra and canakinumab are other medications that may help some children.

Ibuprofen or acetaminophen can be given to relieve pain and fever.

Children tend to outgrow PFAPA syndrome.

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