Management of PFAPA syndrome in patients who fulfill the diagnostic criteria including response to a test dose of glucocorticoids*

PFAPA: periodic fever with aphthous stomatitis, pharyngitis, and adenitis.
* Test dose of glucocorticoids: Give 1 to 2 mg/kg of prednisone or prednisolone orally as a single dose (maximum 60 mg). If a 1 mg/kg dose was given and fever recurs 12 to 48 hours later, a second 1 mg/kg dose may be given. Fever resolution within 6 hours of receiving glucocorticoids is highly suggestive of PFAPA. Patients requiring more than 2 mg/kg per episode should be evaluated for alternate causes of recurrent fever.