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 |