PFAPA Syndrome in Children: Symptoms, Diagnosis and Treatment

Your child has a temperature again. High fever, a sore throat, swollen glands in the neck — and you have the feeling this is exactly what happened last month. And the month before. The short answer first: recurrent fever in toddlers is almost always a run of ordinary infections, and even when PFAPA syndrome really is behind it, this is a benign condition that stops on its own. How to spot the difference, and what your GP needs from you, is set out section by section below.

What is PFAPA syndrome?

PFAPA stands for periodic fever, aphthous stomatitis, pharyngitis and adenitis — recurring bouts of fever together with mouth ulcers, a sore throat and swollen neck glands. It is not an infection and it is not contagious. Among the periodic fever syndromes of childhood, it is the most common one.

Common still means rare here. PRINTO, the European paediatric rheumatology organisation, states that the frequency of PFAPA is not known, but that the condition appears to be more common than generally appreciated. A Norwegian study found around 3.5 new cases per 10,000 children under five per year.

The cause is unknown. PFAPA is not an immune deficiency and not an allergy. It is not inherited in a straightforward way either: familial cases have been described, but no genetic cause has been found. Episodes usually begin before the age of five, most often between two and five.

How do you recognise a PFAPA episode?

Episodes start abruptly, often out of complete health. The temperature rises quickly and high. Alongside it come a sore throat, swollen glands in the neck, and in some children mouth ulcers. After three to six days it is over, as suddenly as it started.

The giveaway is the repetition. Episodes come back every three to six weeks, in many children at strikingly regular intervals. Parents often describe being able to predict the next one from the calendar.

Feature	How it looks in PFAPA
Onset of fever	Sudden, often with shivering
Length of an episode	3 to 6 days
Interval between episodes	About 3 to 6 weeks, often very regular
Throat	Redness and pain on swallowing, almost always present
Glands	Swollen on both sides of the neck, usually tender
Mouth ulcers	In roughly 40 to 70 per cent of children — their absence does not rule PFAPA out
Between episodes	Child completely well, growing and developing normally

Some children also complain of headache, aching limbs or tummy ache. These are not the leading complaints. A rash, conjunctivitis or a swollen joint are not part of the picture.

When does your child still need urgent medical help?

A known PFAPA diagnosis does not protect your child from picking up something else. The usual fever warning signs therefore still apply, whether or not the episode fits the familiar pattern. The NHS defines a high temperature as 38C or above.

If your child is under 3 months old and has a temperature of 38C or higher, or is 3 to 6 months old with a temperature of 39C or higher, get urgent advice from NHS 111 or your GP straight away — and call 999 if they seem seriously unwell.

One step below that sit the situations for a same-day GP call: fever lasting more than five days, a child who is not drinking, or simply the feeling that something is off. NHS 111 is available around the clock for advice when you are not sure.

Is it really PFAPA — or something else?

In the overwhelming majority of cases, recurrent fever is not a syndrome but a toddler at nursery. Paediatric guidance puts 8 to 12 infections a year in the normal range at this age, and when they arrive back to back it feels like a permanent state. We have broken down how many infections are still normal in a separate article.

The difference lies less in the frequency than in the pattern. Ordinary infections turn up irregularly, bring a cough and a runny nose with them, and last different lengths of time. PFAPA episodes run the same way in the same child every time — same symptoms, same duration, similar spacing.

That is exactly why the judgement belongs to a doctor. Cyclic neutropenia, a disorder of the white blood cells, can resemble PFAPA and has to be excluded. Tonsillitis also looks deceptively similar during an episode — we have described when tonsillitis in children actually needs an antibiotic.

How is the diagnosis made?

There is no laboratory test that proves PFAPA. It is a diagnosis of exclusion, built from the clinical picture and from watching several episodes go by. Inflammatory markers such as CRP are checked during an episode and again in the symptom-free interval — in PFAPA they are normal in between.

Four points are generally required together: at least three fever episodes at regular intervals, a sore throat with swollen neck glands or mouth ulcers, complete freedom from symptoms and normal growth between episodes, and a rapid response to a single dose of a steroid.

That last point is why you should never source that steroid yourself. It is given under medical supervision, and specialist paediatric input — rheumatology, immunology or infectious diseases — is normally sought before it is used.

How do you keep track of the episodes?

The pattern is the finding. And that pattern does not emerge in the consulting room — it emerges at home, over months of ordinary evenings. Specialist guidance is unusually blunt about this: confirming the diagnosis requires a carefully kept fever diary, recorded episode by episode as it happens.

Four things matter per episode: the start and end date, the temperatures you measured with the time of day, the accompanying symptoms — throat, glands, ulcers — and every dose of medicine with time and amount. The start and end dates are what give you the interval to the next episode, and that interval is the decisive signal in PFAPA.

In Mona you log a reading in a few seconds and the fever chart builds itself. Over several months that turns into exactly the overview your GP needs: episode by episode, with the gaps between them, instead of a memory of "some time in June". Losing a scrap of paper matters when a diagnosis takes half a year to assemble.

Your GP will ask: when did the last episode start, how long did it last, how high did the temperature go, and was your child genuinely well in between? Those answers are already in your record. We have written about why your memory fails at the GP and what helps.

How is PFAPA syndrome treated?

There is no treatment for the underlying cause, and because the course is benign and self-limiting, any treatment counts as optional. The decision is made case by case, weighing how much the child is suffering against possible side effects. Doing nothing and simply supporting your child through the episodes is a legitimate choice.

For symptoms, paracetamol or ibuprofen can be used. They mainly ease the sore throat and the general feeling of illness; in only a minority of children do they bring the temperature down substantially. Follow the dosing on the packaging and your doctor's instructions, and never exceed the recommended amount. No treatment is given between episodes.

The second option is a single dose of a steroid at the start of an episode, which often ends it within hours. The catch: in roughly a quarter of treated children the symptom-free intervals then get shorter — in one study from five weeks to three. That makes it a case-by-case medical decision rather than a standing solution.

Removing the tonsils is reported in the literature with cure rates above 80 per cent, but it remains contested. Specialist centres do not recommend it as a first step, precisely because PFAPA resolves on its own anyway. Antibiotics do not work — PFAPA is not a bacterial infection.

Do children grow out of PFAPA?

Yes. PFAPA is described as a benign, self-limiting condition that fades over years. Episodes gradually become milder, less frequent and shorter, and in most children they stop altogether during the early primary school years. Spontaneous remission is usually reached by around the age of ten.

No lasting damage is expected. Neither growth nor mental and motor development are affected — that is in fact one of the criteria the diagnosis depends on. Between episodes your child is an ordinary child who can do everything.

Common questions about PFAPA syndrome

Is PFAPA syndrome contagious?

No. PFAPA is not an infectious disease and cannot be passed on, not even during an episode. Siblings and other children at nursery are not at risk. Infections can trigger an episode, though, and an ordinary infection can of course arrive on top of one.

When should I raise PFAPA with my GP?

When your child has had at least three fever episodes that repeated at similar intervals, and was completely well in between. Take your fever diary with you. For each individual episode, the usual fever warning signs apply regardless of any suspected diagnosis.

Can my child go to nursery with PFAPA?

During an episode your child stays at home because they are unwell with a high temperature, not because of any risk to others. As a rule of thumb, wait until your child is fit and free of fever before they go back. Between episodes there are no restrictions at all.

Does my child need special arrangements at school?

Usually not. Because episodes typically stop during the early primary school years, only a few children are affected for long. It is worth telling the nursery or school about the diagnosis, so that recurring absences are not misread as constant infections or as poor attendance.

Can PFAPA occur in adults?

Rarely, but it has been described. The typical onset is between the ages of two and five. A markedly earlier onset can point to a hereditary fever syndrome and should be assessed by a doctor. Paediatric rheumatology or immunology is the right specialty for that judgement.

What if there are no mouth ulcers?

That is common and does not rule PFAPA out. Depending on the study, mouth ulcers are found in roughly 40 to 70 per cent of affected children, and they are often missed because they hurt less than expected. The sore throat is the symptom that is almost always there.

Summary: what to do when

Situation	What to do
Stiff neck, rash that does not fade, difficulty breathing, very hard to wake	Call 999 or go to A&E
Under 3 months with 38C or above, or 3 to 6 months with 39C or above	Urgent — NHS 111 or your GP straight away
Fever lasting more than 5 days, or you are unsure	Contact your GP today, or call NHS 111
Third similar fever episode at a similar interval, well in between	Book a GP appointment and bring your fever diary
Known PFAPA episode following the usual pattern, child drinking and responsive	Comfort, rest, fluids — log the course in Mona