Table 2.

New set of PFAPA classification criteria and Modified Marshall’s classification criteria with number of patients that didn’t fulfill each criterion

New classification criteria	Patients excluded (N)	Modified Marshall’s classification criteria	Patients excluded (N)
1. Periodic Fever for at least 6 months:  a. Daily fever of at least 38.5 °C (axillar) for 2 to 7 days  b. At least 5 regularly recurring fever episodes with maximum of 2 months interval between them	20	Regularly recurring fevers with an early age onset (<  5 years of age)	7
2. Pharyngitis, cervical adenitis, oral aphthae: at least one in every episode and at least 2 out of 3 in the majority of episodes.	21	2. Constitutional symptoms in absence of upper respiratory infection with at least one of the following clinical signs: • apthous stomatitis • cervical lymphadenitis • pharyngitis	0
3. Exclusion of other causes of recurrent fever (clinical or by laboratory depending on history)	0	3. Exclusion of cyclic neutropenia	0
4. Exclusion of infections, immunodeficiency and cyclic neutropenia	0
5. Disease onset before the age of 6 years	1
6. Full recovery between episodes	2	4. Completely asymptomatic interval between episodes	2
7. Normal linear growth	4	5. Normal growth and development	3