Table 4.

Clinical diagnostic criteria resulting from the multivariate analysis.

Diagnostic criteria for adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome
Recurrent fever accompanied by (a)Erythematous pharyngitis and/or (b)Cervical lymphadenitis
Increased inflammatory markers during attacks
Symptom-free intervals

Diagnostic items accounted for their odds ratio (OR) values: variables with a higher OR value (increased inflammatory markers during attacks and symptom-free intervals) were established as mandatory in the final diagnostic model; conversely, on the basis of a lower OR value, only one item between erythematous pharyngitis during fever and cervical lymphadenitis during fever is required for the diagnosis of PFAPA syndrome. These diagnostic criteria should be applied on patients aged at least 16 years and after having excluded infective, autoimmune, and neoplastic diseases as well as monogenic autoinflammatory diseases (AIDs) and febrile polygenic AIDs. In addition, throat swab performed during fever have to be negative and antibiotic therapy ineffective.