Periodic fever, aphthous stomatitis, pharyngitis (PFAPA) syndrome is the most common disorder of the periodic fever syndromes. PFAPA shows up between the ages of 2 and 5 years old. Children with PFAPA have reoccurring fevers, mouth sores, sore throat and swollen lymph nodes in the neck. An episode of PFAPA can last for three to seven days and occurs every three to six weeks. PFAPA can last many years, but it usually goes away between the ages of 10 and 20 years old.
There is not a specific lab test that can diagnose PFAPA. The syndrome will be diagnosed based on symptoms and a physical exam. The child’s white blood cell count will be increased during an episode of PFAPA.
The goal in treating PFAPA is to control symptoms during an episode, to shorten the length of an episode, and increase the amount of time between episodes. With PFAPA, the fever usually does not respond to acetaminophen (Tylenol) or anti-inflammatory medicines like ibuprofen.
Steroids may greatly reduce the length of the episode but could cause the next episode to occur sooner. Certain medications (cimetidine and colchicine), if taken regularly, may stop future episodes in about 1/3 to ½ of children.
Some studies have shown that a tonsillectomy (removing the tonsils by surgery) can cure PFAPA in more than 80% of patients.