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Table 1 Diagnostic criteria used for PFAPA

From: PFAPA syndrome: a review on treatment and outcome

I. Regularly recurring fevers with an early age of onset (<5 years of age)
II. Constitutional symptoms in the absence of upper respiratory infection with at least 1 of the following clinical signs : a) aphtous stomatitis, b) cervical lymphadenitis, c) pharyngitis
III. Completely asymptomatic interval between episodes
IV. Normal growth and development