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PFAPA Syndrome

By

Apostolos Kontzias

, MD, Stony Brook University School of Medicine

Last full review/revision Sep 2021
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Topic Resources

PFAPA 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. Recently it has been recognized in adults as well.

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 of PFAPA Syndrome

  • 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 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 drugs that may help some children.

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

Children tend to outgrow this syndrome.

Drugs Mentioned In This Article

Generic Name Select Brand Names
TYLENOL
TAGAMET
ADVIL, MOTRIN IB
KINERET
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