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What Is Pfapa And Why Does It Matter?, Dennis J. Baumgardner

Dennis J. Baumgardner, MD

PFAPA, the syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical lymphadentitis, is an uncommon disease of episodic immune dysregulation that usually begins in early childhood. Symptoms occur at regular, predictable intervals, with healthy periods in between, and generally include the namesake signs and various other constitutional symptoms such as fever and fatigue. The etiology is unknown and there is no definitive diagnostic test or treatment. Uncertainty during the period of searching for a diagnosis may be disconcerting to the family and the clinician. Single-dose prednisone is effective for resolving the fever of PFAPA episodes within just a few hours, …