Things to Know About PFAPA Syndrome

What You Need to Know About PFAPA Syndrome
Things to Know About PFAPA Syndrome

High fever, which alarms parents and often does not know how to behave, is one of the most common problems in which a doctor is consulted. Frequent repetition of high fever at certain intervals can seriously reduce the quality of life of the child and his family, while negatively affecting the school success of children. A rheumatic disease called PFAPA syndrome can cause this recurrent resistant fever. Acıbadem Altunizade Hospital Pediatrics, Pediatric Rheumatology Specialist Assoc. Dr. Ferhat Demir says that PFAPA Syndrome is one of the most common reasons for unnecessary antibiotic use in children over the age of one. Pediatric Rheumatology Specialist Assoc. Dr. Ferhat Demir explained 9 important points to know about PFAPA syndrome (recurrent fever), which can be seen in all seasons, and made important warnings and suggestions.

Do not give antibiotics because it does not help!

PFAPA syndrome is an additional common rheumatic periodic fever disease of childhood, which usually lasts between 3-6 days and goes away spontaneously, frequently recurring, with persistent fever, pharyngitis, tonsillitis (tonsillitis), mouth sores and lymph node enlargement findings. Assoc. Dr. Ferhat Demir “PFAPA syndrome is not an infection, and it is not a condition that requires antibiotics. It is not contagious. The most common misapplication we see in this disease is that children use antibiotics for unnecessary reasons, sometimes several times a month, considering that they have beta microbes or throat infections.

Watching with these symptoms!

In children, fever, which reaches 3-4 degrees with an interval of 39-40 weeks, develops. The attack range may decrease up to a week or expand to two-three months. The most common finding accompanying fever is the presence of white plaques on the tonsils in the throat. Enlargement of the neck lymph nodes, pharyngitis-tonsillitis, sores in the mouth, joint pain, more rarely rash, abdominal pain and diarrhea may accompany. Between the attacks, the children are completely healthy and there is no effect on growth and development due to the disease.

Can show familial transmission

In PFAPA Syndrome (recurrent fever), attacks often begin between the ages of 2-5 and disappear from the age of 7-8 years. In some patients, these attacks may continue in adolescence and adulthood. Researches; Although a genetic cause cannot be determined exactly, it suggests that the disease may show familial transmission. In our own clinical experience, we can see that in certain patients, such as parents-uncle-aunt-aunt-uncle, there are similar findings in childhood, and the findings cease after tonsillectomy.

It can be seen in all seasons!

One of the features of the disease is that it does not observe the seasons, unlike other infections; Although it is more common in winter and spring, PFAPA attacks can develop in any season. The reason why it is more common in some seasons is that possible viral infections can trigger the PFAPA attack by stimulating the immune system. In this respect, families of children with PFAPA should be more protective and careful about upper respiratory tract infections. Children should not be restricted from their school and social life as long as their general condition is good.

The main reason; intense work of the immune system

Pediatric Rheumatology Specialist Assoc. Dr. Ferhat Demir said, “While the immune system is working intensively in PFAPA syndrome, symptoms similar to infectious diseases may develop and cause patients to receive unnecessary treatments as if they have an infection. "With current scientific data, it's not known for sure what causes it, but we do know that certain genetic conditions can pose a risk for this disease."

It can be confused with other diseases!

The diagnosis of the disease is made by a doctor's examination and the appearance of similar attacks of the patient. In laboratory tests, heights are seen as if there is a microbial condition in the body. Before making a diagnosis of PFAPA, it is necessary to exclude other diseases that may cause similar findings. Because, in addition to other infectious diseases, the findings of FMF, which is common in our country, and a few rheumatic periodic fever syndromes can be confused with PFAPA.

Pay attention to this point in treatment!

Assoc. Dr. Ferhat Demir said, “Although steroid (cortisol) treatment is frequently used and beneficial during relapse periods, an undesirable side effect of steroid application is that it causes shortening of attack intervals. After steroid administration, attacks may become more frequent up to once a week. In this respect, steroid therapy is not a treatment method that we recommend to be used every month or more frequently. For this reason, after the evaluation of the pediatric rheumatologist and exclusion of other rheumatic causes, additional treatments can be given to help reduce the frequency of attacks, if necessary. Adeno-tonsillectomy (nasal and tonsil surgery) is the most effective treatment method that provides complete termination of attacks in 85-90% of patients. There are higher-level treatment options for patients whose symptoms persist and persist despite tonsil surgery.

Constant monitoring is required!

PFAPA does not cause any permanent problems. It does not retard growth and development, but it can cause febrile convulsions due to high fever in children with low seizure threshold. Diagnosed patients should definitely follow up with a pediatric rheumatologist. Since PFAPA disease is basically a rheumatic fever disease, it is strongly recommended that these children be evaluated in terms of other periodic rheumatic fever diseases.

Early diagnosis and treatment is very important!

Assoc. Dr. Ferhat Demir said, “The biggest problem we experience due to the disease is the serious decrease in the quality of life of both the child and the family. This is more prominent especially in children who have attacks once a month or more frequently. For this reason, children's school life may also be interrupted. In this respect, our main goal should be to make a good differential diagnosis in the early period and to reduce or completely eliminate the frequency and severity of attacks with effective treatment.

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