Medical Conditions

PFAPA Syndrome (Periodic Fever) in Children

Medically reviewed by Dr. Michael Okonkwo, MD, FAAP · Board-Certified Neonatologist

Content reviewed against published AAP, NIH, ACR guidelines

Editorial policy

Last reviewed:

If your baby has been diagnosed with or you suspect pfapa syndrome (periodic fever) in children, here is what the evidence says.

The short answer

PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis) is the most common periodic fever syndrome in children. It causes high fevers (often 104-105°F) that recur like clockwork every 3-6 weeks, lasting 3-5 days each time. During episodes, children may also have mouth sores (aphthous ulcers), sore throat (pharyngitis), and swollen neck lymph nodes (adenitis). Between episodes, children are completely well and growing normally. PFAPA is not caused by infection and responds dramatically to a single dose of prednisone. Most children outgrow PFAPA within several years.

Key takeaways

  • PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis) is the most common periodic fever syndrome in children. It causes high fevers (often 104-105°F) that recur like clockwork every 3-6 weeks, lasting 3-5 days each time. During episodes, children may also have mouth sores (aphthous ulcers), sore throat (pharyngitis), and swollen neck lymph nodes (adenitis). Between episodes, children are completely well and growing normally. PFAPA is not caused by infection and responds dramatically to a single dose of prednisone. Most children outgrow PFAPA within several years.
  • Usually normal when: Your child is completely healthy, active, and growing normally between fever episodes
  • Call your doctor if: Your child has a high fever and appears severely ill, is difficult to arouse, or has signs of a serious infection — even children with PFAPA can develop real infections that need urgent treatment
  • Varies by age — see the age-by-age breakdown below
“Fever itself is not an illness — rather, it is a sign or symptom that the body is fighting an infection. Fever stimulates certain defenses, such as the white blood cells, which attack and destroy invading bacteria.”
— Fever and Your Child, American Academy of Pediatrics (AAP)

This is one of the most common questions parents ask. Searching for answers means you care.

What Parents Should Know

According to AAP, NIH, ACR guidelines, pFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis) is the most common periodic fever syndrome in children. It causes high fevers (often 104-105°F) that recur like clockwork every 3-6 weeks, lasting 3-5 days each time. During episodes, children may also have mouth sores (aphthous ulcers), sore throat (pharyngitis), and swollen neck lymph nodes (adenitis). Between episodes, children are completely well and growing normally. PFAPA is not caused by infection and responds dramatically to a single dose of prednisone. Most children outgrow PFAPA within several years. At 0-6 months, pFAPA is very rare before age 1 and typically does not present in infancy. Periodic fevers in a baby under 6 months require careful evaluation for other causes, including infections, primary immunodeficiency, and other autoinflammatory conditions. If your baby has recurrent fevers, your pediatrician should evaluate thoroughly rather than attributing them to PFAPA. It is generally considered normal when your child is completely healthy, active, and growing normally between fever episodes. However, you should contact your pediatrician promptly if your child has a high fever and appears severely ill, is difficult to arouse, or has signs of a serious infection — even children with PFAPA can develop real infections that need urgent treatment.

Sources: [1], [2], [3]

Normal vs. Concerning

Usually Normal
Worth Discussing
Your child is completely healthy, active, and growing normally between fever episodes
Your child has a high fever and appears severely ill, is difficult to arouse, or has signs of a serious infection — even children with PFAPA can develop real infections that need urgent treatment
Fever episodes follow a regular, predictable pattern every 3-6 weeks
Your child has periodic fevers along with hearing loss, joint swelling, rash, or severe abdominal pain, which may indicate a different autoinflammatory condition requiring different treatment
Your child's fever responds dramatically to a single dose of corticosteroid, typically resolving within hours
Your child has a pattern of recurring fevers every few weeks and you want to discuss whether PFAPA could be the cause
Lab tests between episodes are entirely normal, including inflammatory markers
Your child has been diagnosed with PFAPA and episodes are becoming more frequent or steroids are needed more often

By Age

What to expect by age

0-6 months

PFAPA is very rare before age 1 and typically does not present in infancy. Periodic fevers in a baby under 6 months require careful evaluation for other causes, including infections, primary immunodeficiency, and other autoinflammatory conditions. If your baby has recurrent fevers, your pediatrician should evaluate thoroughly rather than attributing them to PFAPA.

6-12 months

PFAPA can occasionally begin late in the first year but is uncommon. The typical onset is between 2 and 5 years of age. If your baby has a pattern of recurring fevers, keep a fever diary documenting the dates, duration, and associated symptoms. This pattern information is the most valuable diagnostic tool. Other periodic fever syndromes (like Familial Mediterranean Fever) and other causes should be considered at this age.

1-3 years

This is the most common age of onset for PFAPA. Parents often notice a predictable pattern — high fever appearing every 3-6 weeks, lasting 3-5 days, with the child completely well between episodes. During fevers, the child may have mouth sores, sore throat (often with negative strep tests), and swollen lymph nodes in the neck. Importantly, growth and development are normal between episodes. A hallmark of PFAPA is the dramatic response to a single dose of oral corticosteroid (prednisone or prednisolone), which typically breaks the fever within hours.

3+ years

PFAPA can continue through later childhood, though most children outgrow it, with the average resolution around age 8-10. Treatment options include corticosteroids given at the onset of each episode (which stop the fever but may shorten the interval between episodes), cimetidine (which can prevent episodes in some children), and tonsillectomy, which resolves PFAPA in approximately 60-90% of children. PFAPA is not associated with long-term health consequences, and children grow and develop normally.

What to Tell Your Pediatrician

  • Describe when you first noticed pfapa syndrome (periodic fever) in children and how it has changed over time.
  • Note your baby's current age and which age-specific patterns you are seeing.
  • Mention if your child has a pattern of recurring fevers every few weeks and you want to discuss whether PFAPA could be the cause.
  • Mention if your child has been diagnosed with PFAPA and episodes are becoming more frequent or steroids are needed more often.
  • Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
  • Bring a list of any questions or observations you want to discuss at the appointment.

What Should You Do?

When to take action

Probably normal when...
  • Your child is completely healthy, active, and growing normally between fever episodes
  • Fever episodes follow a regular, predictable pattern every 3-6 weeks
  • Your child's fever responds dramatically to a single dose of corticosteroid, typically resolving within hours
  • Lab tests between episodes are entirely normal, including inflammatory markers
Mention at your next visit when...
  • Your child has a pattern of recurring fevers every few weeks and you want to discuss whether PFAPA could be the cause
  • Your child has been diagnosed with PFAPA and episodes are becoming more frequent or steroids are needed more often
  • You want to discuss whether tonsillectomy might be an appropriate option for your child with PFAPA
  • Your child with periodic fevers also has joint pain, rash, chest pain, or abdominal pain, which may suggest a different periodic fever syndrome
Act now when...
  • Your child has a high fever and appears severely ill, is difficult to arouse, or has signs of a serious infection — even children with PFAPA can develop real infections that need urgent treatment
  • Your child has periodic fevers along with hearing loss, joint swelling, rash, or severe abdominal pain, which may indicate a different autoinflammatory condition requiring different treatment

What You Can Do at Home

  • Keep track of when you notice pfapa syndrome (periodic fever) in children — noting the time of day, duration, and any triggers can help your pediatrician.
  • Remember that your child is completely healthy, active, and growing normally between fever episodes — this is generally within the range of normal.
  • At 0-6 months, focus on observation rather than intervention unless your pediatrician advises otherwise.
  • Follow any care instructions from your pediatrician. Keep a written log of symptoms to bring to appointments.
  • While monitoring at home, seek immediate care if your child has a high fever and appears severely ill, is difficult to arouse, or has signs of a serious infection — even children with PFAPA can develop real infections that need urgent treatment.

My Baby Has a Fever That Won't Go Away

Most fevers in babies and toddlers are caused by viral infections and resolve within 3-5 days. A fever that lasts longer than 3 days, returns after seeming to resolve, or is accompanied by worsening symptoms warrants medical evaluation. The most important thing is how your baby looks and acts - a child who is alert and drinking well with a fever is generally less concerning than one who is listless, regardless of the temperature.

Getting Sick Again and Again: Illness Clustering

It is completely normal for babies and toddlers, especially those in group care settings, to get sick 8 to 12 times per year - and these illnesses often cluster in fall and winter, making it feel like your child is constantly unwell. Each infection helps build their immune system. However, if illnesses seem unusually frequent, severe, or slow to resolve, it is worth discussing with your pediatrician to rule out underlying conditions.

Signs of Primary Immunodeficiency in Babies

Primary immunodeficiency disorders are conditions where the immune system does not function properly from birth. Warning signs include 4 or more new ear infections in a year, 2 or more serious sinus infections in a year, 2 or more months on antibiotics with little effect, 2 or more pneumonias in a year, failure to thrive, recurrent deep skin or organ abscesses, and a family history of primary immunodeficiency. These conditions are rare but treatable when identified early.

Triage

Fever Decision Tree

Step-by-step guidance for evaluating your baby's fever by age and temperature.

Milestone

Month-by-Month Development

Detailed monthly development guides from birth through 24 months.

Guide

When to Call the Doctor

General guide on when to call the pediatrician, visit urgent care, or go to the ER.

Frequently asked questions

Is pfapa syndrome (periodic fever) in children normal?
PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis) is the most common periodic fever syndrome in children. It causes high fevers (often 104-105°F) that recur like clockwork every 3-6 weeks, lasting 3-5 days each time. During episodes, children may also have mouth sores (aphthous ulcers), sore throat (pharyngitis), and swollen neck lymph nodes (adenitis). Between episodes, children are completely well and growing normally. PFAPA is not caused by infection and responds dramatically to a single dose of prednisone. Most children outgrow PFAPA within several years.
When should I call the doctor about pfapa syndrome (periodic fever) in children?
Your child has a high fever and appears severely ill, is difficult to arouse, or has signs of a serious infection — even children with PFAPA can develop real infections that need urgent treatment Your child has periodic fevers along with hearing loss, joint swelling, rash, or severe abdominal pain, which may indicate a different autoinflammatory condition requiring different treatment
When is pfapa syndrome (periodic fever) in children normal?
Your child is completely healthy, active, and growing normally between fever episodes Fever episodes follow a regular, predictable pattern every 3-6 weeks Your child's fever responds dramatically to a single dose of corticosteroid, typically resolving within hours
What causes pfapa syndrome (periodic fever) in children?
PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis) is the most common periodic fever syndrome in children. It causes high fevers (often 104-105°F) that recur like clockwork every 3-6 weeks, lasting 3-5 days each time. During episodes, children may also have mouth sores (aphthous ulcers), sore throat (pharyngitis), and swollen neck lymph nodes (adenitis). Between episodes, children are completely well and growing normally. PFAPA is not caused by infection and responds dramatically to a single dose of prednisone. Most children outgrow PFAPA within several years. Common explanations include: Your child is completely healthy, active, and growing normally between fever episodes. Fever episodes follow a regular, predictable pattern every 3-6 weeks.
What should I mention to my pediatrician about pfapa syndrome (periodic fever) in children?
You should mention pfapa syndrome (periodic fever) in children at your next visit if: Your child has a pattern of recurring fevers every few weeks and you want to discuss whether PFAPA could be the cause. Your child has been diagnosed with PFAPA and episodes are becoming more frequent or steroids are needed more often. You want to discuss whether tonsillectomy might be an appropriate option for your child with PFAPA.
Is pfapa syndrome (periodic fever) in children normal at 0-6 months?
PFAPA is very rare before age 1 and typically does not present in infancy. Periodic fevers in a baby under 6 months require careful evaluation for other causes, including infections, primary immunodeficiency, and other autoinflammatory conditions. If your baby has recurrent fevers, your pediatrician should evaluate thoroughly rather than attributing them to PFAPA.
Is pfapa syndrome (periodic fever) in children normal at 6-12 months?
PFAPA can occasionally begin late in the first year but is uncommon. The typical onset is between 2 and 5 years of age. If your baby has a pattern of recurring fevers, keep a fever diary documenting the dates, duration, and associated symptoms. This pattern information is the most valuable diagnostic tool. Other periodic fever syndromes (like Familial Mediterranean Fever) and other causes should be considered at this age.
Should I go to the ER for pfapa syndrome (periodic fever) in children?
Seek emergency care if your child has a high fever and appears severely ill, is difficult to arouse, or has signs of a serious infection — even children with PFAPA can develop real infections that need urgent treatment, or if your child has periodic fevers along with hearing loss, joint swelling, rash, or severe abdominal pain, which may indicate a different autoinflammatory condition requiring different treatment. When in doubt, call your pediatrician's after-hours line for guidance.
Does pfapa syndrome (periodic fever) in children go away on its own?
In many cases, pfapa syndrome (periodic fever) in children resolves on its own, especially when your child is completely healthy, active, and growing normally between fever episodes. By 3+ years, pFAPA can continue through later childhood, though most children outgrow it, with the average resolution around age 8-10. Treatment options include corticosteroids given at the onset of each episode (which stop the fever but may shorten the interval between episodes), cimetidine (which can prevent episodes in some children), and tonsillectomy, which resolves PFAPA in approximately 60-90% of children. PFAPA is not associated with long-term health consequences, and children grow and develop normally.
AAPNIHACR

References

  1. [1]American Academy of Pediatrics. PFAPA Syndrome. Pediatrics in Review, 2019. AAP
  2. [2]National Institute of Allergy and Infectious Diseases. Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA). Genetic and Rare Diseases Information Center, 2023. NIH
  3. [3]American College of Rheumatology. Autoinflammatory Diseases. ACR, 2023. ACR

Doctor Visit Checklist

Bring this checklist to your next pediatrician visit to discuss PFAPA Syndrome (Periodic Fever) in Children.

Things to mention

  • Describe when you first noticed pfapa syndrome (periodic fever) in children and how it has changed over time.
  • Note your baby's current age and which age-specific patterns you are seeing.
  • Mention if your child has a pattern of recurring fevers every few weeks and you want to discuss whether PFAPA could be the cause.
  • Mention if your child has been diagnosed with PFAPA and episodes are becoming more frequent or steroids are needed more often.
  • Let your doctor know if you have noticed any related concerns, such as changes in feeding, sleep, or movement patterns.
  • Bring a list of any questions or observations you want to discuss at the appointment.

Observations to share

  • Your child has a pattern of recurring fevers every few weeks and you want to discuss whether PFAPA could be the cause
  • Your child has been diagnosed with PFAPA and episodes are becoming more frequent or steroids are needed more often
  • You want to discuss whether tonsillectomy might be an appropriate option for your child with PFAPA

Urgent signs to report immediately

  • Your child has a high fever and appears severely ill, is difficult to arouse, or has signs of a serious infection — even children with PFAPA can develop real infections that need urgent treatment
  • Your child has periodic fevers along with hearing loss, joint swelling, rash, or severe abdominal pain, which may indicate a different autoinflammatory condition requiring different treatment

My notes

From ismybabyalright.com — free, evidence-based baby health guides

All content follows our editorial policy and is reviewed against published clinical guidelines.

2,705 evidence-based guides6 authoritative medical sources5 medical advisory board members

Related Resources

Fever TriageWell-Baby Visit PrepMedicine Dosage CalculatorBaby Tracker

Bottom line

Most cases of pfapa syndrome (periodic fever) in children are normal. Talk to your pediatrician if your child has a high fever and appears severely ill, is difficult to arouse, or has signs of a serious infection — even children with pfapa can develop real infections that need urgent treatment.

Trust your instincts. If something feels wrong, reach out to your pediatrician. Worrying about your baby means you care — that is a good thing.

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Most fevers in babies and toddlers are caused by viral infections and resolve within 3-5 days. A fever that lasts longer than 3 days, returns after seeming to resolve, or is accompanied by worsening symptoms warrants medical evaluation. The most important thing is how your baby looks and acts - a child who is alert and drinking well with a fever is generally less concerning than one who is listless, regardless of the temperature.

Getting Sick Again and Again: Illness Clustering

It is completely normal for babies and toddlers, especially those in group care settings, to get sick 8 to 12 times per year - and these illnesses often cluster in fall and winter, making it feel like your child is constantly unwell. Each infection helps build their immune system. However, if illnesses seem unusually frequent, severe, or slow to resolve, it is worth discussing with your pediatrician to rule out underlying conditions.

Signs of Primary Immunodeficiency in Babies

Primary immunodeficiency disorders are conditions where the immune system does not function properly from birth. Warning signs include 4 or more new ear infections in a year, 2 or more serious sinus infections in a year, 2 or more months on antibiotics with little effect, 2 or more pneumonias in a year, failure to thrive, recurrent deep skin or organ abscesses, and a family history of primary immunodeficiency. These conditions are rare but treatable when identified early.

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