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PFAPA Syndrome

By

Apostolos Kontzias

, MD, Stony Brook University School of Medicine

Medically Reviewed Sep 2021 | Modified Sep 2022
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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
Acid Reducer, Major Acid Reducer, Tagamet, Tagamet HB
Kineret
Ilaris
Advil, Advil Children's, Advil Children's Fever, Advil Infants', Advil Junior Strength, Advil Migraine, Caldolor, Children's Ibuprofen, ElixSure IB, Genpril , Ibren , IBU, Midol, Midol Cramps and Body Aches, Motrin, Motrin Children's, Motrin IB, Motrin Infants', Motrin Junior Strength, Motrin Migraine Pain, PediaCare Children's Pain Reliever/Fever Reducer IB, PediaCare Infants' Pain Reliever/Fever Reducer IB, Samson-8
7T Gummy ES, Acephen, Aceta, Actamin, Adult Pain Relief, Anacin Aspirin Free, Apra, Children's Acetaminophen, Children's Pain & Fever , Comtrex Sore Throat Relief, ED-APAP, ElixSure Fever/Pain, Feverall, Genapap, Genebs, Goody's Back & Body Pain, Infantaire, Infants' Acetaminophen, LIQUID PAIN RELIEF, Little Fevers, Little Remedies Infant Fever + Pain Reliever, Mapap, Mapap Arthritis Pain, Mapap Infants, Mapap Junior, M-PAP, Nortemp, Ofirmev, Pain & Fever , Pain and Fever , PAIN RELIEF , PAIN RELIEF Extra Strength, Panadol, PediaCare Children's Fever Reducer/Pain Reliever, PediaCare Children's Smooth Metls Fever Reducer/Pain Reliever, PediaCare Infant's Fever Reducer/Pain Reliever, Pediaphen, PHARBETOL, Plus PHARMA, Q-Pap, Q-Pap Extra Strength, Silapap, Triaminic Fever Reducer and Pain Reliever, Triaminic Infant Fever Reducer and Pain Reliever, Tylenol, Tylenol 8 Hour, Tylenol 8 Hour Arthritis Pain, Tylenol 8 Hour Muscle Aches & Pain, Tylenol Arthritis Pain, Tylenol Children's, Tylenol Children's Pain+Fever, Tylenol CrushableTablet, Tylenol Extra Strength, Tylenol Infants', Tylenol Infants Pain + Fever, Tylenol Junior Strength, Tylenol Pain + Fever, Tylenol Regular Strength, Tylenol Sore Throat, XS No Aspirin, XS Pain Reliever
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