Periodic Fever with Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA)

What is Periodic Fever with Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA)?

Some children have frequent fevers when they are otherwise healthy. One cause of frequent fevers is a condition called Periodic Fever with Aphthous stomatitis, Pharyngitis and cervical Adenitis (PFAPA). Children with PFAPA usually start having fevers before age 5 years of age. Fevers usually happen regularly every 2-8 weeks and they may have canker sores (aphthous stomatitis), sore throat (pharyngitis), and swollen bumps in the neck (cervical adenitis).  These are called episodes. Children with PFAPA may not have all these symptoms with every episode. Between fevers and symptoms, children are healthy with otherwise normal growth. PFAPA can run in families, but does not always. In most children, PFAPA gets better on its own, on average in about 3 years. Although the symptoms can interfere with life and be uncomfortable during episodes, PFAPA does not appear to harm children.

How is PFAPA diagnosed?

Your healthcare team will review your child’s symptoms and laboratory testing to rule out the many other important conditions.  Keep a diary of your child’s fevers including dates and symptoms they experience during fever to help determine which laboratory or imaging tests they need.  Testing varies, but can include testing blood, urine, and performing imaging tests like chest x-rays. Sometimes your provider may test when your child is feeling well and when feeling sick.

How is PFAPA treated?

Your child’s healthcare provider will help determine which option is best for your child. There are several options to managing PFAPA, including:

• Providing comfort during episodes- PFAPA does not lead to long-term health problems in children, so the fever episodes are not harmful. Giving medicines like acetaminophen (Tylenol®) or anti-inflammatory medications, like ibuprofen (Motrin®) during episodes may help your child to feel comfortable. 
• Your provider may prescribe a steroid medicine given by mouth at the start of a fever episode. This stops the episode of fever. For some children who take steroids, the time between fevers may shorten.  
• Daily medicines, such as colchicine and cimetidine, can help lower the number of episodes. These medicines are sometimes used if other options have not worked.
• Removing your child’s tonsils (tonsillectomy) stops fever episodes all together in many children. Because of the risks of surgery, tonsillectomy may be an option after discussing closely with your child’s provider.  

If my child does not have PFAPA, what happens next?

Some children may have frequent fevers and many viral infections.  For them, no treatment is needed. For children without many viral infections, genetic testing and a referral to doctor who specializes in conditions of joints, muscles, and ligaments (Rheumatologist).

Questions?

This information is not specific to your child but provides general information. If you have any questions, please call your clinic.

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