PFAPA (periodic fever, aphthous stomatitis, pharyngitis and adenitis) in children and adolescent – recurrent fever with sore throat

PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis) in children and adolescent – recurrent fever with sore throat

PFAPA is the most common syndrome causing recurrent fever in children. There is a great importance to be familiar with this syndrome, since an accurate diagnosis will prevent many unnecessary antibiotic treatments, and will shorten episodes of fever and discomfort in those children.
I am aware that PFAPA is under the section of “infectious diseases”, but this is an inflammatory disease and not infectious disease. Since it imitates streptococcal throat infections (link), I chose to tag this post under infectious diseases.

What does PFAPA stands for?

The acronym is a list of the disease’ manifestations, as follow:
Periodic fever: typically recurring every few weeks
Aphthous: some cases present with aphthous (shallow ulcers) in the pharynx
Pharyngitis: inflammation of the tonsils
Adenitis: enlargement of lymph nodes (lymphadenopathy), mainly cervical.

What is the common age for PFAPA?

Presentation is mainly in ages of 2-5 years of age, even though this syndrome has been described in every age, including young adults.

What are the clinical manifestations of PFAPA?

Recurrent episodes of fever every 3-6 weeks.

How does every episode of PFAPA look like?

Fever, fatigue, pharyngitis with exudates on the tonsils, cervical lymphadenopathy and sometimes mouth sores. Additional complaints may present – headaches, abdominal pain, and arthralgia.

How long does an episode of PFAPA last without treatment?

About 4-6 days, with or without antipyretics or antibiotics (given unnecessarily).

What is the cause for PFAPA? What is the difference between infection and inflammation?

PFAPA is an example of inflammation, and not for infection. Think of a local trauma or back spasm for example which cause local inflammation, and there are no bacteria or viruses involved in that process.
The trigger for the inflammatory process in PFAPA is not clear. It is possible that genetic factors cause an inadequate response of our immune system to an unknown trigger.
Remember, that since it is not a disease caused by bacteria, antibiotic therapy will not suffice.

How will the doctor diagnose PFAPA?

On one hand it is very easy – recurrent events of fever and pharyngitis in children. There are almost no other situations similar to that.
On the other hand, the pediatrician needs time perspectives and high index of suspicious in order to identify this syndrome. It is almost impossible to suspect PFAPA in the first and even the second event.
And on retrospect – you know these adults who tell about repeated strep pharyngitis as children? Those who received penicillin shots or went through tonsillectomy because of repeated events? Well, many of them had PFAPA.

What do you find in a throat examination during a PFAPA episode?

Every episode of PFAPA will cause white exudates on the tonsils. It may not be seen on the first or second day, but eventually, on a good physical examination – it will be found.
In my opinion, every pharyngitis with exudates must be sampled for strep’ (you can use this link to read more of strep’ throat). You can use quick strep’ swab or a throat culture. If the swab is negative, the suspicion for PFAPA will be high. If it is positive, the doctor must ask himself if this is a true strep’ infection justifying antibiotic treatment or just a carrier state (as for about 15% of the population).

What to do if the child already has a diagnosis of PFAPA and now starts a new pharyngitis? How do we differentiate the two?

First of all, children and their parents usually know how to tell the difference. Though supposedly there is no difference between the two (strep’ pharyngitis or PFAPA), there are still specific characteristics for each child and his mother usually can tell the difference. On the other hand, a child with PFAPA can have strep’ pharyngitis, like every other child. So in any cases where the answer is not clear (for example, an episode arriving earlier than the usual pattern), a throat culture or swab must be taken (and not be confused with carriers of strep’). You can read more about the specific characteristics of strep pharyngitis in this post.

What will happen to a child with strep’ pharyngitis, receiving a treatment for PFAPA with steroids (as you will see ahead)

He will get better for 12 hours and then the fever will return.

What is the treatment for an episode of PFAPA?

The treatment is a onetime dose of steroids, followed by a dramatic improvement of symptoms in hours. As for the dosage and the type of steroids – it’s up to the child, his weight and his ability to swallow pills. In most cases, since it more prevalent in young children, a type of steroids which dissolves in water will be recommended (Betamethasone).

What is the appropriate steroid dosage for treatment?

We must differentiate the first time the child is treated from the next times. The first time has a diagnostic significance. Since the dramatic effect of steroids confirms the diagnosis, it’s a shame to miss it if given inappropriate dosage.
So in the first event, a high dose of Betamethasone of 0.3 milligram per kilogram of body weight will be administered.
After the diagnosis has been made, next events will warrant a lower dose of steroids. The goal is, by trial and error, to learn the lowest dosage of steroids that “gets the job done”.

What do you mean by “gets the job done”?

This means stopping an episode of PFAPA, so the next episode will appear after at least 7 days. Here lays the problem if we treat with a low dose of steroids – the episode will continue and will return in less than 7 days. This is mostly a sign of an inadequate dose of steroids (or a wrong diagnosis).

Is there any damage in treating with high dose of steroids?

Usually there are no series side effects or any damage when treating with a onetime dose in this dosage in every episode. Steroids have side effects when they are given for many consecutive days. One of the known side effects of Betamethasone is restlessness in children. In these children we must find the dose that ends the episode but does not cause restlessness. In cases where this does not work, you can choose another steroid with your physician (one that does not cross the blood brain barrier as much as Betamethasone).
The disease itself or the steroid treatment does not cause any harm to the child.

Are there any other treatments for PFAPA?

In stubborn cases who do not respond to steroids, you can consult with an expert. There are some attempts in treating with other drugs like cimetidine, singulair or colchicine. Some try tonsillectomy.

What will happen to children with PFAPA? When will it pass?

Episodes frequency lower as the child grows up. After a few years the disease passes. An interesting anecdote is that sometimes after the diagnosis is made and appropriate treatment is started, the time length between episodes shortens for a while but then returns back to the usual pattern

Does it worth treating PFAPA? It’s just a fever for e few days

PFAPA is unpleasant for the child, even if not dangerous. 3-6 days of high fever is not something I wish for any child. I recommend trying treating with steroids in these children. The benefit for the child from a single dose of steroids is substantial.

In conclusion, it still makes me wonder in the year of 2022, when all the information is available, how so many PFAPA cases are being missed. It’s true that it is easy to miss the first 1-2 episodes, but after a few events that happen every few weeks, the suspicion should be high.
This syndrome does not leave any damages for the long term, and treatment, annoying as it may be, is simple and leads to a quick recovery. A simple and optimistic pediatric medicine.

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