PFAPA Syndrome

What is PFAPA Syndrome?

PFAPA syndrome is a childhood disease that affects both boys and girls. It is a disease characterized by recurrent attacks of fever, swelling of the lymph nodes in the throat, sore throat and mouth ulcers. PFAPA usually begins in early childhood, between the ages of 2 and 5.

What does PFAPA stand for?

PFAPA syndrome stands for; "Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis".

What Causes PFAPA?

The cause of the disease is unknown. During febrile periods, the immune system is activated. This activation causes an inflammatory response that includes fever and inflammation of the mouth or throat. Although familial cases have been described, a genetic cause has not been found to date.

What are the symptoms of PFAPA?

The main symptom is recurrent fever accompanied by sore throat, mouth ulcers, or enlarged lymph nodes in the neck. Fever attacks begin suddenly and last for 3 to 6 days. Fever attacks sometimes recur every 3-6 weeks, with extremely regular intervals. Between attacks, the child's activity is normal and healthy between attacks.

There is no specific laboratory test or imaging method for the diagnosis of PFAPA. The disease is diagnosed by evaluating physical examination and laboratory tests together. It is imperative to exclude all other diseases that exhibit similar symptoms before the diagnosis can be confirmed.

How is PFAPA Diagnosed?

Since there is no specific laboratory test or imaging method for the diagnosis of PFAPA, the disease is diagnosed by evaluating physical examination and laboratory tests together. During attacks, the values of tests such as erythrocyte sedimentation rate (ESR, sediment) and C-reactive protein (CRP) levels in the blood increase. Before the diagnosis can be confirmed, other diseases that cause recurrent fever with similar symptoms must be excluded.

How is PFAPA Treated?

There is no disease-specific treatment that completely cures PFAPA syndrome. The aim of treatment is to control symptoms during fever attacks. In the majority of cases, symptoms decrease as the child grows and disappear on their own. During fever attacks, antipyretics can reduce the fever to some extent. It is known that a single dose of prednisone, given when symptoms first begin, reduces fever and shortens the duration of the attack. However, with this treatment, the time between attacks may be shortened and the next febrile attack may occur earlier than expected. If the quality of life of the child and family is seriously affected due to recurrent fever attacks, tonsil removal may be considered in some patients.

What is the Prognosis of PFAPA Disease?

In the long term, as the child grows, PFAPA usually disappears on its own or becomes less serious. The child's growth and development are usually not affected by this disease.