Recurrent Fever in Children May Signal PFAPA Syndrome

Recurrent Fever in Children May Signal PFAPA Syndrome One of the most common complaints doctors receive is recurrent high fever in children. The cause of persistent resistant fevers could be a rheumatological disease called PFAPA syndrome. Assoc. Prof. Dr. Ferhat Demir, Pediatrics and Pediatric Rheumatology Specialist at Altunizade Hospital, shared valuable information about PFAPA syndrome. Demir noted that this syndrome causes resistant fevers and is among the most frequent reasons for unnecessary antibiotic use in children. Demir shared 9 important points to know about PFAPA syndrome.

What Should Be Known About PFAPA Syndrome

High fever is one of the leading reasons children visit doctors. Assoc. Prof. Dr. Ferhat Demir, Pediatrics and Pediatric Rheumatology Specialist at Acıbadem Altunizade Hospital, said that PFAPA syndrome may be one of the causes of recurrent high fever in children. PFAPA syndrome is a frequently occurring rheumatological periodic fever disease in childhood, characterized by episodes lasting typically 3-6 days that resolve spontaneously, recurrent resistant fever, pharyngitis, tonsillitis (tonsil inflammation), mouth ulcers and lymph node enlargement. Demir stated: "PFAPA syndrome is not an infection, and it is certainly not a condition requiring antibiotics. It is not contagious. The most common incorrect practice we observe in this disease is that children are thought to have strep bacteria or throat infection, sometimes leading to unnecessary antibiotic use several times a month."

PFAPA Syndrome Causes These Symptoms

Information about the symptoms of PFAPA syndrome was also shared. According to the information provided, the disease develops in children with fever complaints reaching 39-40 degrees Celsius at 3-4 week intervals, and the interval between attacks can decrease to one week or extend to two-three months. In addition to these complaints, enlargement of neck lymph nodes, pharyngitis-tonsillitis, mouth ulcers, joint pain, and more rarely, rash, abdominal pain and diarrhea may also be observed.

The Disease Can Show Familial Transmission

It was noted that attacks in PFAPA syndrome typically begin between ages 2-5 and disappear from age 7-8 onwards. In some patients, these attacks may continue into adolescence and adulthood. According to information shared by Demir, research suggests that while not establishing a definitive genetic cause, the disease can show familial transmission. Demir noted that in his clinical experience, he has observed similar findings in childhood among relatives such as parents, uncles and aunts.

The Main Cause Is Intense Activity of the Immune System

It was shared that the main cause of PFAPA syndrome is intense activity of the immune system. Demir stated: "In PFAPA syndrome, while the immune system works intensely, findings similar to infectious diseases can develop and may lead to patients receiving unnecessary treatment as if they had an infection. Although it is not definitively known what causes this with current scientific data, we know that certain genetic conditions can create risk for this disease."

Information Was Also Provided Regarding Treatment of the Disease

Assoc. Prof. Dr. Ferhat Demir shared information about treatment of the disease: "While steroid (cortisol) therapy is frequently used during attack periods and shows benefit, an undesirable side effect of steroid therapy is that it causes intervals between attacks to shorten. Following steroid therapy, attacks can increase to weekly frequency. From this perspective, steroid therapy is not a treatment method we recommend for use monthly or more frequently. For this reason, after pediatric rheumatology specialist evaluation rules out other rheumatological causes, additional treatments that may help reduce attack frequency can be given if necessary. Adenotonsillectomy (removal of adenoids and tonsils) is the most effective treatment method, providing complete cessation of attacks in 85-90 percent of patients. In patients whose attacks continue despite tonsillectomy and follow a resistant course, higher-level treatment options are available."

Early Diagnosis and Treatment Are Very Important in the Course of the Disease

Assoc. Prof. Dr. Ferhat Demir emphasized the importance of early diagnosis and treatment, stating: "The greatest difficulty we experience related to this disease is a significant reduction in quality of life for both the child and the family. This is especially observed in the foreground in children experiencing attacks monthly or more frequently. For this reason, the child's school life may also be interrupted. From this perspective, our main goal should be to perform good differential diagnosis in the early period and through effective treatment reduce or completely eliminate the frequency and severity of attacks." Demir also emphasized that patients with a diagnosis must be followed by a pediatrician.