Periodic Fever, Aphthous Stomatitis, Pharyngitis, Cervical Adenitis (PFAPA) is a syndrome that consists of recurrent episodes of fever, sore throat, mouth sores and swelling of the glands in the neck. The frequency of PFAPA is not known, but the disease appears to be more common than originally thought, and may be the most common recurrent fever (autoinflammatory) syndrome that does not come from an infection. Both males and females and all ethnic groups can develop PFAPA. PFAPA usually starts in early childhood, between the ages of two to five years. Occasionally, PFAPA may develop at an older age, including rare cases in adults.
It is not yet known what causes this disorder. No gene defect has yet to be found in PFAPA, although sometimes more than one family member has the disease and a history of a tonsillectomy (surgical removal of the tonsils) may be reported among family members. No infection has been found in PFAPA, and it is not a contagious disease. It is clear that the inflammatory process is active during episodes, but it is not clear why this happens.