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Table 2 New set of PFAPA classification criteria and Modified Marshall’s classification criteria with number of patients that didn’t fulfill each criterion

From: Towards a new set of classification criteria for PFAPA syndrome

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