Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) Syndrome: An Overview for Primary Care Practitioners

Abstract

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common autoinflammatory disease (AID) that causes recurrent fever in children. Patients with PFAPA present with essential symptoms such as periodic fever (usually every 28 days) and the PFAPA triad (stomatitis, pharyngitis, and adenitis) and additional symptoms such as abdominal pain, headache, rash, or arthralgia. PFAPA is diagnosed by (1) demonstrating the clinical manifestations included in the PFAPA criteria and (2) excluding other diseases that cause recurrent fever in children, such as recurrent infections and/or allergic diseases, other AIDs including monogenic hereditary recurrent fever (HRF) and syndrome of undifferentiated recurrent fever (SURF), and cyclic neutropenia. Genetic investigations may be necessary for patients with recurrent but irregular fever, predominant additional symptoms, growth and developmental delay, or worsening of symptoms after age 10. Treatment for PFAPA includes supportive care, medications (steroids, cimetidine, or colchicine), and surgical therapy (tonsillectomy). Treatment modalities should be determined on the basis of the benefits and side effects of the treatment and parental preferences. PFAPA may be more common in children with recurrent fever than previously thought. On the other hand, some children may be mistaken for PFAPA or may undergo special testing for PFAPA simply because of their frequent fever patterns. Therefore, an overview of PFAPA is necessary not only for specialists but also for primary care practitioners.