Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome (PFAPA) (Juvenile)
Periodic Fever, Aphthous Stomatitis, Pharyngitis, Cervical Adenitis, or PFAPA, is a syndrome that usually affects children. PFAPA usually starts between ages two and five. Males and females of any ethnic group can get PFAPA. PFAPA is an autoinflammatory disease, but its cause is unknown. It is not triggered by infection and is not contagious. In about 15% of patients, PFAPA persists into adulthood with occasional episodes. Episodes usually become less frequent over time. There is no gene linked to PFAPA, but it sometimes runs in families. Inflammation is involved in PFAPA, but it’s unclear why this happens. Children with PFAPA grow and develop normally.
What Are the Signs/Symptoms?
PFAPA causes recurrent fevers with mouth sores (stomatitis) and red, sore throat (pharyngitis). Children may have white patches on their tonsils and swollen lymph glands in the neck (adenitis). Other possible signs are joint pain, rash, vomiting, diarrhea, headache, or stomach pain. Fevers start suddenly and last for 3-7 days. Episodes may occur every 3 to 6 weeks with children completely well between episodes. A doctor will diagnose PFAPA with a physical examination. There is no laboratory test for PFAPA, but two common markers of inflammation (C-reactive protein and sedimentation rate) rise during the fevers. Other causes for the fevers, such as Streptococcus infection – especially if symptoms start in the child’s first year of life - must be first ruled out. Prompt reaction to steroid treatment may help confirm PFAPA.
What Are Common Treatments?
The goals of PFAPA treatment are to ease symptoms, shorten the duration of fevers and prevent recurrence. These fevers usually don’t respond well to over-the-counter treatments like acetaminophen (Tylenol) or ibuprofen (Advil, Motrin). When symptoms first appear, one dose of steroids like prednisone or prednisolone usually shortens or ends the episode. However, steroids may shorten time between episodes. Children who need steroids more often than every 3 to 4 weeks may need other treatments. Tonsillectomy (surgery to remove tonsils) cures PFAPA in more than 80% of children, but the best timing for this treatment is unclear. In many children, other medications like cimetidine and colchicine may prevent future PFAPA episodes.
Living with PFAPA
While PFAPA may cause children to miss days of school, the syndrome isn’t a long-term danger to the physical health of the child. PFAPA usually resolves spontaneously during the second decade of life. Do not delay setting up a pediatrician appointment if the child shows signs of PFAPA. Seek care from a pediatric rheumatologist for treatment and ongoing management.
Updated February 2023 by Nina T. Washington, MD, MPH, and reviewed by the American College of Rheumatology Committee on Communications and Marketing.
This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.