PFAPA- Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome

PFAPA stands for periodic fever (fever episodes occurring about every 3-6 weeks), aphthous stomatitis (canker sores/mouth ulcers), pharyngitis (sore throat), and adenitis (swollen lymph nodes in the neck). It is important to note, however, that only about 40-50% of patients with PFAPA have ulcers, pharyngitis, and adenitis. Sometimes kids also notice headaches, nausea, vomiting, and/or abdominal pain. It is the most common fever syndrome in children seen in North America. It does not have any known genetic cause and usually is seen in kids younger than 5 years old. Often this disease improves over time and kids often grow out of it by age 11. PFAPA is not contagious.

What else should I know about PFAPA?

There is no specific lab test to diagnosis PFAPA. Often rheumatology providers will ask parents to keep a fever diary, keeping track of when fever episodes occur, what the temperature is, what other symptoms their child is experiencing, etc. These fever diaries can help providers look for patterns in the fever episodes and help determine a diagnosis in addition to the physical exam and blood work.

It is common for kids with PFAPA to first go to their PCP to get throat cultures checked as sore throats and fever are common symptoms. Typically, if a child has PFAPA, these throat cultures will come back “negative” and not show any active infection, such as strep throat. It is also typical for medications such as NSAIDs (Advil, Motrin, etc.) to not be as effective at treating the symptoms of PFAPA.

While there is no specific treatment for PFAPA, providers aim to control the symptoms. Sometimes PFAPA resolves on its own over time. Often, providers may recommend a single dose of a steroid (prednisone/prednisolone) during the beginning of a fever episode, which should stop the fever within hours. It is important to note that by giving these one-time steroid doses at the beginning of a fever episode, that while they can shorten/stop the episode, about 10-20% of kids start to have the fever episodes more frequently when using one-time steroids as a treatment. Another consideration for treatment is a tonsillectomy/adenoidectomy (surgery to take out the tonsils/adenoids), which can help improve or completely resolve the symptoms in 60-80% of patients. Speak to your child’s provider to help determine what treatment plan is right for your child with PFAPA.

Where can I read more about PFAPA?

You can learn more about PFAPA through the QR code below, which takes you to the website for information on pediatric rheumatic diseases (PRINTO).

                                                                 

                                                                                 PFAPA (ACR)

 

If you have any questions, please message us on MyChart or call the care team on 212-263-9969.

 

 

 

Created in partnership with families and expertise from our Sala Institute for Child and Family Centered Care.

 

 

©2025 NYU Langone Health. All rights reserved. Reviewed for health literacy. This information is not intended as a substitute for professional medical care. Always follow your health care provider's instructions.