PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome causes recurring episodes of fever that last 3 to 6 days, mouth sores (stomatitis), a sore throat (pharyngitis), and swollen lymph nodes in the neck (cervical adenitis).
PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome is the most common periodic fever syndrome in children.
PFAPA syndrome typically starts between 2 and 5 years of age and tends to be more common among boys. It has been recognized in adults as well.
Symptoms of PFAPA Syndrome
PFAPA syndrome causes fatigue, chills, and occasionally abdominal pain and headache as well as fever, sore throat, mouth ulcers, and swollen lymph nodes.
About once a month, children have a fever that typically lasts 3 to 6 days.
Children are healthy between episodes, and growth is normal.
Diagnosis of PFAPA Syndrome
A doctor's evaluation
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 steroids (also called glucocorticoids), colchicine, cimetidine, tonsillectomy, or a combinationSometimes steroids (also called glucocorticoids), colchicine, cimetidine, tonsillectomy, or a combination
PFAPA syndrome does not always need to be treated, but children may be given steroids 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.Anakinra and canakinumab are other medications that may help some children.
Ibuprofen or acetaminophen can be given to relieve pain and fever.Ibuprofen or acetaminophen can be given to relieve pain and fever.
Episodes of PFAPA syndrome gradually become less severe, frequent, and shorter in duration over time. Although some children who have PFAPA syndrome may continue to experience symptoms in adolescence, most children stop having them by age 10.
Drugs Mentioned In This Article
