PFAPA (periodic fever, aphtous stomatitis, pharyngitis and cervical adenitis) syndrome registry: analysis of a cohort of 214 patients

A multi-centre cohort of children with PFAPA syndrome is herein presented. The challenges associated with the discovery of pathogenetic pathways in PFAPA syndrome are immense, reflected by the small number of studies currently in progress. As new mechanisms underlying the regulation of innate immunity are discovered and novel therapeutic approaches are adopted, the next decade promises many exciting advances to manage and treat patients with this disorder.