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 (adenitis).
PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome is the most 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. 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
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.
Children tend to outgrow PFAPA syndrome.
