What is PFAPA?
PFAPA is a condition that causes repeated episodes of fever in children.
The name PFAPA describes the main symptoms:
- Periodic Fever
- Aphthous stomatitis (mouth ulcers)
- Pharyngitis (sore throat)
- Adenitis (swollen glands in the neck)
PFAPA is described as “the most common condition” among the autoinflammatory periodic fever syndromes (the group of disorders that cause regular, predictable fever cycles) Other periodic fever syndromes (e.g., FMF, TRAPS) are much rarer, making PFAPA the leading diagnosis within that category.
It usually begins between 2 and 5 years of age and is seen slightly more often in boys.
Most children grow out of PFAPA, although a small number may continue to have symptoms as adults.
PFAPA is not an infection, not contagious, and cannot be treated with antibiotics
What Happens in PFAPA?
Children with PFAPA experience repeated fever episodes, often called flares.
- Typical fever pattern - Fever ranges from 38.5°C to 41°C
- Episodes last 4-5 days
- Flares return every 3-6 weeks
- Children are completely well between episodes
Other symptoms during a flare
- Sore throat
- Tonsillitis
- Mouth ulcers or canker sores
- Swollen glands in the neck
- Abdominal pain
- Nausea or vomiting
- Diarrhoea
- Leg aches or joint pain (without long‑term damage)
- Tiredness or low energy
- Headaches
- Chills
- Febrile seizures (in some children)
- Rashes (uncommon)
PFAPA can look similar conditions. Doctors may investigate further if symptoms are not typical of PFAPA and may suggest another diagnosis e.g. frequent rashes or hives, ongoing symptoms between flares, lung or breathing infections, symptoms starting in the first months of life, protein in the urine, poor growth or development
What Causes PFAPA?
The exact cause of PFAPA is unknown. There is no confirmed genetic marker for PFAPA at this time. A family history of similar symptoms may be relevant, but PFAPA does not follow a clear inheritance pattern. Doctors often check for other periodic fever conditions before diagnosing PFAPA and may do genetic testing for other known autoinflammatory syndromes. If genetic testing does not show a known variant, it does not rule out an autoinflammatory condition.
Many people with symptoms of autoinflammatory disease have normal genetic results.
For example: Around 25–30% of people with Familial Mediterranean Fever have no identifiable variation, yet still respond to treatment. 40–60% of people with systemic autoinflammatory diseases are diagnosed as uSAID (undefined systemic autoinflammatory disease).
There is no single test for PFAPA.
Diagnosis is based on:
- A clear pattern of repeated fevers
- Onset before 5 years of age
- At least one of the following during episodes:
- Mouth ulcers, Swollen neck glands, Sore throat, Normal health and growth between episodes, No signs of infection during flares
Laboratory tests
Blood tests may be done during and between episodes to check inflammation.
Inflammation should rise during a flare and return to normal afterwards.
Tests include:
- Full blood count (FBC)
- C‑reactive protein (CRP)
- Erythrocyte sedimentation rate (ESR)
- Serum amyloid A (SAA)
There is no cure, but several treatments can reduce symptoms or prevent flares.
Symptom relief
Used to reduce fever or discomfort: Paracetamol, Ibuprofen. These medicines may help your child feel better but do not stop the flare.
Abortive treatment
A short course of corticosteroid (steroids) at the start of a flare can stop the fever within a few hours and shorten the episode, but mouth ulcers may take longer to settle and some children may flare more often after steroid use.
Preventive (prophylactic) treatment
Colchicine - Daily medicines may reduce how often flares occur, but they do not work for everyone. Cimetidine - is no longer regularly used in Australia and Aotearoa New Zealand.
Surgical treatment
If flares are frequent or do not respond to medicines, some children may benefit from Tonsillectomy (removal of tonsils) and/or Adenoidectomy (removal of adenoids). Many children have fewer or no flares after surgery.
It is important to discuss options with your health professional and the risks and benefits.
IMPORTANT NOTE:
Currently, not all medications commonly used to treat SAIDs are available in Australia and New Zealand, and those available may be difficult to access.
ANZFAID is committed to continuing to advocate for improved options, and timely and affordable access to treatment.
Children may miss school during flares. Because PFAPA is chronic and episodic, families often work with the school to create:
1. A health plan explaining the condition.
- The student will need to provide a medical letter confirming PFAPA diagnosis.
- Expected symptoms
- When the child should stay home
- Medication used (e.g., corticosteroids if prescribed)
2. A communication protocol for flare days.
3. How the school should record absences - Flexibility for late arrivals or partial attendance.
4. How the child will catch up on missed work or reasonable adjustments to help support school work being done.
Ideas of Reasonable adjustments that may be made depending on individual needs suitable:
- Reduced workload during recovery days
- Access to catch‑up materials
- Extra time for assignments
- Modified PE participation during recovery
- Access to classwork online
Australia does not have one single “chronic illness education law,” but every state and territory is legally required to support students with chronic medical conditions under a combination of federal disability legislation and state/territory education policies.
Aotearoa New Zealand law requires schools to support students with chronic or long‑term medical conditions under the Education and Training Act 2020. Chronic illness is a fully valid reason for justified absence, and schools must provide equitable access to education, reasonable adjustments, and safe health‑management procedures. Education and Training Act 2020 - Ministry of Education
Keeping a symptom diary can help track patterns and flares.
Living with PFAPA
PFAPA does not cause long‑term health problems. Using steroids may have long term complications. Most children outgrow it by late childhood. A small number may continue to have symptoms as adults, but usually less often. Growth and development remain normal. Children can participate in normal activities between episodes. Schools and childcare centres may benefit from a simple explanation of the condition.
Helpful info:
Consensus Treatment Plans for PFAPA
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