PFAPA (periodic fevers with aphthous stomatitis, pharyngitis, and adenitis) syndrome is a periodic fever syndrome that typically manifests between ages 2 yr and 5 yr; it is characterized by febrile episodes lasting 3 to 6 days, pharyngitis, aphthous ulcers, and adenopathy. Etiology and pathophysiology are undefined.
PFAPA syndrome is a relatively common periodic fever among children. Although genetic causes have not been determined, this syndrome tends to be grouped with hereditary fever syndromes. It typically starts in early childhood (between ages 2 yr and 5 yr) and tends to be more common among males.
Febrile episodes last 3 to 6 days and recur about every 28 days. The syndrome causes fatigue, chills, and occasionally abdominal pain and headache, as well as fever, pharyngitis, aphthous ulcers, and lymphadenopathy. Patients are healthy between episodes, and growth is normal.
Diagnosis is based on clinical findings, which include the following:
Acute-phase reactants (eg, C-reactive protein, ESR) are elevated during a febrile episode but not between episodes. Neutropenia or other symptoms (eg, diarrhea, rash, cough) are not present; their presence suggests a different disorder.
Treatment is optional; it can include glucocorticoids, cimetidine, and, rarely, tonsillectomy. Patients tend to outgrow this syndrome without sequelae.
Last full review/revision February 2013 by Stephen E. Goldfinger, MD