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• Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).
• Auto-inflammatory disorder occurring in affected children every 3-8 weeks.
• Onset around age 2; lasting into second decade of life.
• A current pathophysiologic theory is PFAPA is a disorder of the innate immune system involving interleukins, chemoattractants, and cytokines that stimulating the inflammatory response causing symptoms associated with PFAPA.
• Early diagnosis of PFAPA may help prevent the unnecessary use of antibiotics.
• Biomarker CD64, galectin-3 and the MPV may be useful in the diagnosis of PFAPA along with other laboratory values during the febrile attack period of PFAPA.
• Traditional and current treatment of PFAPA is single dose of corticosteroids at onset of symptoms.
• Treatment with tonsillectomy was found to have an 80% cure rate.
• No statistical difference between corticosteroid and tonsillectomy treatments.
• Treatment of PFAPA with antibiotics was found ineffective.
• Treatment of PFAPA with vitamin D, was found by one study to have promising results.
Physician Assistant Studies
Master of Physician Assistant Studies (MPAS)
Fever; Pharyngitis; Stomatitis, Aphthous; Hereditary Autoinflammatory Diseases; Child
Infectious Disease | Pediatrics | Rheumatology
Lahti, Jessica, "PFAPA: A Periodic Febrile Syndrome Afflicting Children" (2015). Physician Assistant Scholarly Project Posters. 98.