PFAPA Syndrome: How Many Fevers Are Too Many?

When my daughter was diagnosed with PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) syndrome, I wasn’t surprised.  I myself had suffered from a mysterious recurrent fever in my childhood, and at some point in medical school, I pinpointed PFAPA as the most likely cause. 

But I was surprised to discover just how common it actually is.  Did you know that PFAPA is the most common type of recurrent fever syndrome in children?

As a pediatrician, counseling parents about fever management is about as bread-and-butter as it comes. And we all know that when kids start preschool, we can expect fevers and colds galore. So how can you tease out which of those children aren’t just fighting off a typical virus?  When is “one more fever” just too much? 

Children with PFAPA have an auto-inflammatory condition that presents as recurrent febrile illnesses along with aphthous ulcers, adenitis and/or pharyngitis. Here are 3 clinical pearls to help you tease out when you might want to start thinking about PFAPA:

• Episodes happen at regular intervals - Children with PFAPA often start to have regular fevers around age 2-5, and those fevers occur at predictable intervals. It can be anywhere from 2-8 weeks, most commonly every 4-5 weeks. That interval between episodes can be so predictable that some families can predict the precise day when a child will develop a fever! (This is a handy feature when planning family vacations.
  
  
• No one else at home is sick  - A funny thing happened when the world shut down at the start of the COVID pandemic. With school germs eliminated as a factor, there was suddenly no reason why children with monthly fevers were the only member of the family still getting sick every month. There were multiple reports of spikes in PFAPA diagnoses during the lockdown period of the COVID pandemic, proving that it’s more common than we might think. 
  
  
• A child has been diagnosed with repeated cases of “tonsillitis” - but with strep tests consistently negative or not checked. Since a febrile episode can look a lot like strep throat, some children can present as recurrent tonsillitis, but that’s why it’s so important to make sure a strep test has been done. This is helpful to keep in mind when asking about family history: about 40% of children with PFAPA have a family member with the condition. But instead of frequent fevers, a parent might recall recurrent tonsillitis. 

Thankfully, modern medicine has evolved from the time of my childhood, when the original paper describing PFAPA had yet to be published. Back then I was simply doomed to miss out on class events every month and wait out my 4-5 days of fever with apple juice and tv. 

Nowadays, we have a number of potential treatment options. While there are many treatments available (check out this month’s Peds RAP episode to learn more), 2 of the most noteworthy interventions are:

• Oral steroids: prednisone (1mg/kg/dose) given as a one-time dose early in the fever course can stop the febrile episode in its tracks. The full resolution of fever in response to a dose of steroid is so dramatic that it can actually be used as a diagnostic point in the newer PFAPA criteria. 
  
  
• Tonsillectomy: while the decision to proceed with tonsillectomy needs to be made carefully and with full consideration of the risks and benefits, the data on tonsillectomy is impressive. Some studies show a 90+% rate of cure. 

PFAPA typically resolves itself around the time of adolescence. But the symptoms can lead to considerable distress and disruption in family life. (My daughter had no less than 15 febrile episodes in her first year of fevers!) For our family, getting treatment was life-changing.

Listen to this month’s Peds RAP episode on PFAPA on the Peds RAP podcast to arm yourself with all the tools you’ll need to confidently diagnose and guide these kiddos to the treatment they need.  See our full chapter summary here.