What is PFAPA Syndrome?

Repeated fever attacks – the PFAPA syndrome is considered a rare disease that usually only occurs in childhood. The mysterious disease has so far hardly been researched.

The rare disease PFAPA syndrome occurs primarily in children under the age of five. The term stands for periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis. Translated roughly: recurring fever, sores in the oral mucosa, sore throat and swollen lymph nodes in the neck area.

These are also the main symptoms, with bouts of fever accompanied by very high temperatures and recurring at intervals of four to six weeks, sometimes two to twelve weeks. In addition, abdominal and muscle pain or skin rashes can also occur.

Causes of PFAPA syndrome

To this day they are unclear. In the affected children, inflammatory markers and autoantibodies can be detected in the blood during the acute phase, which can indicate an autoimmune disease.

An autoimmune disease is caused by a dysregulation of the immune system. The immune system attacks the body’s own tissue.

However, in the case of PFAPA syndrome, it is not clear which endogenous structures the immune system is attacking.

How is PFAPA syndrome treated?

Antibiotics are often prescribed for young patients, but they are useless in this disease. However, cortisone, an artificially produced hormone, is showing success, above all the fever episodes seem to be slowed down by the administration. The agent has an anti-inflammatory, anti-allergic effect and inhibits the body’s immune system.

In the phases of illness, the children should drink a lot, especially water, tea or diluted fruit juices. Calf compresses can relieve the high fever. At temperatures above 39.5 degrees, a doctor should be informed, then antipyretics can be used.

What’s the prognosis?

On average, children suffer from the disease for around two and a half to eight years. The PFAPA syndrome usually disappears by itself during childhood, and long-term effects can usually be ruled out. Only in rare cases do patients continue to suffer from the symptoms as adults.