Periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndromePPeriodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndromePeriodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndromeEnglishRheumatologyToddler (13-24 months);Preschooler (2-4 years);School age child (5-8 years);Pre-teen (9-12 years)Mouth;NeckImmune systemConditions and diseasesCaregivers Adult (19+)NA2015-07-15T04:00:00ZRonald M. Laxer, MDCM, FRCPC8.0000000000000061.0000000000000893.000000000000Health (A-Z) - ConditionsHealth A-Z<p>Information about the symptoms, causes, diagnosis and treatment of periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndrome.</p><h2>What is periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndrome?</h2> <p>Periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndrome causes sudden attacks of high fever. These attacks occur on a regular basis. Children with PFAPA can also have swollen glands, sore throat and/or sores in the mouth. High fever on a regular basis is called periodic fever. Swollen glands are called adenitis. Sore throat is called pharyngitis. Sores in the mouth are called aphthous ulcers.</p> <p>Sometimes children do not complain of these other symptoms. A doctor should check whether these symptoms are present with the fever.</p><h2>Key points</h2> <ul> <li>PFAPA is a disease that causes regular attacks of fever.</li> <li>The attacks are accompanied by swollen neck glands, sore throat and/or sores in the mouth.</li> <li>PFAPA can be treated with medications or removal of the tonsils.</li> <li>PFAPA will go away in almost all children by the early teen years.</li> </ul><h2>Symptoms of PFAPA</h2><p>The major symptom of PFAPA is fever. The episodes of fever occur every three to six weeks, while the fever itself usually lasts about three to five days. Children may complain of pain in the throat and they may find it hard to swallow because of this pain. They may also complain of pain in the mouth because of mouth ulcers. The glands in the neck may be enlarged. Children seem quite ill during attacks. However, they recover completely between attacks.</p><p>The attacks eventually stop by late childhood. There are no long-term effects. Children with PFAPA grow and develop normally.</p> <br><h2>Causes of PFAPA</h2> <p>The cause of PFAPA is not yet known. For reasons that are not yet understood, the body’s inflammation system becomes active, leading to the symptoms of PFAPA.</p> <p>PFAPA is not caused by an infection that we know about. Children are not contagious when they have a fever from PFAPA. PFAPA is not hereditary. It is not common for more than one child in a family to have it.</p><h2>Diagnosis of PFAPA</h2> <p>It takes time to diagnose PFAPA. Usually infection is suspected at first. Antibiotics are often prescribed with the first few attacks. After a while, the frequency of the attacks and the similarity of the episodes make the physician and the parents suspect that infection is not the cause.</p> <p>There are no specific tests to diagnose PFAPA. However, many tests are often done to exclude other causes of fevers. Blood tests should be done when the child has fever and when the child is well. In a child who has PFAPA, the test results should come back to normal between attacks.</p><h2>Treatment of PFAPA</h2> <p>Although PFAPA does not have any long-term consequences, most children (and families) are quite disturbed by the frequency of episodes and by how badly the children feel during the attacks. Antibiotics do not work at all. The fevers are only partly controlled with medications like <a href="/Article?contentid=62&language=English">acetaminophen</a> or <a href="/Article?contentid=153&language=English">ibuprofen</a>. The fever responds better to ibuprofen than acetaminophen. Fever medication does not shorten the length of the attacks.</p> <p>There is no medication that can cure PFAPA. Prednisone given at the start of an attack can shorten the length of the attack quite a bit. Sometime two doses, 24 hours apart, are needed. However, the attacks may happen a little more often if prednisone has been used. Seeing how children do with <a href="/Article?contentid=221&language=English">prednisone</a> treatment does help confirm the diagnosis of PFAPA.</p> <p>A newer medicine, anakinra, given once per day by injection into the skin is also very effective. It is only given during the episode until the fever stops.</p> <p>Cimetidine, a drug given twice a day, may work in some patients.</p> <p><a href="/Article?contentid=193&language=English">Montelukast</a>, a drug for <a href="/Article?contentid=785&language=English">asthma</a> given once a day, may also work in some children.</p> <p>Another treatment that has been successful is removal of tonsils and adenoids. It is not clear why this works.</p> <p>In some children, attacks may return after several years.</p>
Syndrome PFAPA (syndrome caractérisé par une fièvre périodique accompagnée d’une stomatite aphteuse, d’une pharyngite et d’une adénite cervicale)SSyndrome PFAPA (syndrome caractérisé par une fièvre périodique accompagnée d’une stomatite aphteuse, d’une pharyngite et d’une adénite cervicale)Periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndromeFrenchRheumatologyToddler (13-24 months);Preschooler (2-4 years);School age child (5-8 years);Pre-teen (9-12 years)Mouth;NeckImmune systemConditions and diseasesCaregivers Adult (19+)NA2015-07-15T04:00:00ZRonald M. Laxer, MDCM, FRCPC8.0000000000000061.0000000000000893.000000000000Health (A-Z) - ConditionsHealth A-Z<p>Renseignements sur les symptômes, les causes, le diagnostic et le traitement du syndrome PFAPA</p><h2>En quoi consiste le syndrome PFAPA (syndrome de fièvre périodique accompagnée d’une stomatite aphteuse, d’une pharyngite et d’une adénite cervicale)?</h2><p>PFAPA (pour Periodic fever adenitis pharyngitis aphthous ulcer) veut dire : syndrome de fièvre périodique avec stomatite aphteuse, pharyngite et adénite cervicale. Le syndrome provoque régulièrement des poussées de forte fièvre (fièvre périodique). Celles-ci peuvent être accompagnées de ganglions enflés (adénite), d’un mal de gorge (pharyngite) ou d’ulcérations dans la bouche (stomatite aphteuse).</p><p>Comme les enfants ne se plaignent parfois pas de ces symptômes, un médecin doit déterminer si les poussées de fièvre périodiques en sont accompagnées.</p><h2>À retenir</h2><ul><li>Le PFAPA est une maladie qui déclenche des poussées de fièvre périodiques</li><li>Les poussées de fièvre sont accompagnées de ganglions enflés dans le cou, d’un mal de gorge ou de lésions dans la bouche</li><li>Le PFAPA peut être traité à l’aide de médicaments ou par l’ablation (extraction) des amygdales</li><li>Le PFAPA disparaît chez presque tous les enfants qui en sont atteints dès le début de l’adolescence.</li></ul><h2>Les symptômes du syndrome PFAPA</h2><p>La fièvre constitue le plus important symptôme du syndrome PFAPA. Les poussées de fièvre surviennent à des intervalles réguliers de 3 à 6 semaines, tandis que la fièvre même dure environ 3 à 5 jours. Les enfants peuvent alors se plaindre d’un mal de gorge, lequel peut leur donner des difficultés à avaler. Ils peuvent aussi se lamenter sur les douleurs à la bouche causées par les aphtes. Les ganglions du cou peuvent aussi enfler. Les enfants semblent alors très malades, bien qu’ils se rétablissent entièrement entre les épisodes.</p><p>Ces épisodes finissent par s’arrêter pendant l’enfance avancée. Le syndrome PFAPA n’a aucune incidence à long terme. Chez les enfants touchés, la croissance et le développement se font normalement.</p><br><h2>Cause du syndrome PFAPA</h2> <p>La cause du PFAPA reste indéterminée. Pour des motifs qui demeurent inexpliqués, le système inflammatoire se met d’abord en branle, puis les symptômes apparaissent.</p> <p>Le PFAPA n’est pas causé par une infection connue. Les enfants qui présentent une fièvre liée au syndrome ne sont pas contagieux. Le PFAPA n’est pas héréditaire. Enfin, il arrive rarement que plus d’un enfant d’une même famille en soit atteint.</p><h2>Diagnostic de PFAPA</h2> <p>Il faut du temps afin de diagnostiquer le PFAPA. Au départ, on soupçonne habituellement une infection et les médecins prescrivent souvent des antibiotiques lors des premières manifestations. Au bout d’un moment, toutefois, le médecin et les parents mettent en doute l’infection compte tenu de la fréquence et de la similitude des épisodes.</p> <p>Bien que bon nombre d’examens soient fréquemment réalisés afin d’exclure les causes connues de la fièvre, aucun d’entre eux ne permet de diagnostiquer le PFAPA. Des analyses de sang doivent être faites pendant que la fièvre est présente de même que lorsque l’enfant se porte bien. Chez les enfants atteints du PFAPA, les examens effectués entre les épisodes devraient donner des résultats négatifs.</p><h2>Traitement du PFAPA</h2><p>Même si le PFAPA n’a aucune répercussion à long terme, la fréquence et l’intensité des épisodes inquiètent les enfants et leurs familles. Les antibiotiques n’ont aucun effet sur le syndrome. Tant l’acétaminophène (Tylenol) que l’ibuprofène (Advil et Motrin) ne permettent que de maîtriser partiellement la fièvre, quoique ce dernier donne de meilleurs résultats. Les médicaments ciblant la fièvre en particulier ne permettent pas d’abréger les épisodes.</p><p>Il n’existe aucun médicament pouvant guérir le PFAPA. La prednisone peut réduire de beaucoup la durée des épisodes à condition que l’enfant la prenne dès que l’épisode est enclenché. Cela peut parfois exiger la prise de deux doses à 24 heures d’intervalle. En revanche, l’usage de la prednisone peut accroître légèrement la fréquence des épisodes. L’efficacité du traitement à la prednisone facilite la confirmation du diagnostic de PFAPA.</p><p>Un nouveau médicament, l’anakinra, qui est donné par injection sous-cutanée est aussi très efficace. On ne l’utilise que pendant l’épisode jusqu'à ce que la fièvre disparaisse.</p><p> La prise deux fois par jour de la cimetidine, peut être bénéfique chez certains enfants.</p><p>Une dose quotidienne de montelukast, un médicament prescrit aux personnes atteintes d’asthme, peut aussi donner de bons résultats chez un certain nombre d'enfants.</p><p>Un autre bon traitement est l’ablation (extraction) des amygdales et des végétations adénoïdes, bien qu’on ne sache pas bien à quoi est due son efficacité.</p><p>On observe que le syndrome peut parfois se déclencher de nouveau plusieurs années après qu’il ai pris fin.<br></p>

 

 

Periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndrome921.000000000000Periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndromePeriodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndromePEnglishRheumatologyToddler (13-24 months);Preschooler (2-4 years);School age child (5-8 years);Pre-teen (9-12 years)Mouth;NeckImmune systemConditions and diseasesCaregivers Adult (19+)NA2015-07-15T04:00:00ZRonald M. Laxer, MDCM, FRCPC8.0000000000000061.0000000000000893.000000000000Health (A-Z) - ConditionsHealth A-Z<p>Information about the symptoms, causes, diagnosis and treatment of periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndrome.</p><h2>What is periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndrome?</h2> <p>Periodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndrome causes sudden attacks of high fever. These attacks occur on a regular basis. Children with PFAPA can also have swollen glands, sore throat and/or sores in the mouth. High fever on a regular basis is called periodic fever. Swollen glands are called adenitis. Sore throat is called pharyngitis. Sores in the mouth are called aphthous ulcers.</p> <p>Sometimes children do not complain of these other symptoms. A doctor should check whether these symptoms are present with the fever.</p><h2>Key points</h2> <ul> <li>PFAPA is a disease that causes regular attacks of fever.</li> <li>The attacks are accompanied by swollen neck glands, sore throat and/or sores in the mouth.</li> <li>PFAPA can be treated with medications or removal of the tonsils.</li> <li>PFAPA will go away in almost all children by the early teen years.</li> </ul><h2>Symptoms of PFAPA</h2><p>The major symptom of PFAPA is fever. The episodes of fever occur every three to six weeks, while the fever itself usually lasts about three to five days. Children may complain of pain in the throat and they may find it hard to swallow because of this pain. They may also complain of pain in the mouth because of mouth ulcers. The glands in the neck may be enlarged. Children seem quite ill during attacks. However, they recover completely between attacks.</p><p>The attacks eventually stop by late childhood. There are no long-term effects. Children with PFAPA grow and develop normally.</p> <br><h2>Causes of PFAPA</h2> <p>The cause of PFAPA is not yet known. For reasons that are not yet understood, the body’s inflammation system becomes active, leading to the symptoms of PFAPA.</p> <p>PFAPA is not caused by an infection that we know about. Children are not contagious when they have a fever from PFAPA. PFAPA is not hereditary. It is not common for more than one child in a family to have it.</p><h2>Diagnosis of PFAPA</h2> <p>It takes time to diagnose PFAPA. Usually infection is suspected at first. Antibiotics are often prescribed with the first few attacks. After a while, the frequency of the attacks and the similarity of the episodes make the physician and the parents suspect that infection is not the cause.</p> <p>There are no specific tests to diagnose PFAPA. However, many tests are often done to exclude other causes of fevers. Blood tests should be done when the child has fever and when the child is well. In a child who has PFAPA, the test results should come back to normal between attacks.</p><h2>Treatment of PFAPA</h2> <p>Although PFAPA does not have any long-term consequences, most children (and families) are quite disturbed by the frequency of episodes and by how badly the children feel during the attacks. Antibiotics do not work at all. The fevers are only partly controlled with medications like <a href="/Article?contentid=62&language=English">acetaminophen</a> or <a href="/Article?contentid=153&language=English">ibuprofen</a>. The fever responds better to ibuprofen than acetaminophen. Fever medication does not shorten the length of the attacks.</p> <p>There is no medication that can cure PFAPA. Prednisone given at the start of an attack can shorten the length of the attack quite a bit. Sometime two doses, 24 hours apart, are needed. However, the attacks may happen a little more often if prednisone has been used. Seeing how children do with <a href="/Article?contentid=221&language=English">prednisone</a> treatment does help confirm the diagnosis of PFAPA.</p> <p>A newer medicine, anakinra, given once per day by injection into the skin is also very effective. It is only given during the episode until the fever stops.</p> <p>Cimetidine, a drug given twice a day, may work in some patients.</p> <p><a href="/Article?contentid=193&language=English">Montelukast</a>, a drug for <a href="/Article?contentid=785&language=English">asthma</a> given once a day, may also work in some children.</p> <p>Another treatment that has been successful is removal of tonsils and adenoids. It is not clear why this works.</p> <p>In some children, attacks may return after several years.</p><h2>How you can help your child with PFAPA</h2> <p>Typical episodes of PFAPA should not be treated with antibiotics. However, children with PFAPA may also get usual childhood infections. If you are concerned that a fever episode is not PFAPA, always contact your child’s doctor.</p> <p>Notify your child’s school about the diagnosis. Children with PFAPA are not contagious. They should not be excluded from school.</p> <p>Children with PFAPA should be encouraged to carry on with normal activities.</p><h2>Follow-up care at the rheumatology clinic</h2> <p>Your child may be referred to a specialty clinic such as rheumatology for follow-up care. Usually, a rheumatology clinic will have a team of doctors and nurses who have a lot of experience treating children with PFAPA.</p> <p>Other members of the rheumatology team include:</p> <ul> <li>a social worker and a child life specialist to help with emotional and behavioural problems that might result from the disease</li> <li>a dietitian to help with your child’s diet and nutrition</li> </ul> <h3>Preparing for visits to the rheumatology clinic</h3> <p>Your child may need a blood test during a clinic visit. This will help doctors monitor the disease. Blood tests also help check for side effects of the medicine your child may be taking. If your child needs to prepare for clinic visits in any other way, a member of the rheumatology team will tell you before the visit.</p> <p>After you have met the rheumatology team, you will know more about how to care for your child and how to plan for future clinic visits.</p>https://assets.aboutkidshealth.ca/AKHAssets/Periodic_fever_adenitis_pharyngitis_aphthous_ulcer_PFAPA_syndrome.jpgPeriodic fever adenitis pharyngitis aphthous ulcer (PFAPA) syndrome

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