Do we need the PFAPA syndrome in adults with non-monogenic periodic fevers?

We read with great interest the article by Gattorno et al proposing a new set of criteria for the classification of autoinflammatory recurrent fevers.1 This year’s Paediatric Rheumatology INternational Trials Organisation(PRINTO) criteria are the third set of criteria for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome in 3 years.1–3 While these three different sets share common points, they also include distinct clinical features, thus resulting in discrepancies in the classification of patients. To illustrate this issue, we report the clinical characteristics of a cohort of 34 consecutive adult patients (see table 1) followed in our centre between 2010 and 2018, and diagnosed with PFAPA based on the modified Marshall’s criteria4 (available as online supplementary material) with the exclusion of age at onset. …