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The PFAPA syndrome is a non-mendelian autoinflammatory disease of unknown aetiology characterized by Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis. In typical cases, attacks begin before the age of five and occur every 2-8 weeks, often with striking periodicity. We have previously profiled the blood cells and serum cytokine levels in a cohort study of patients with PFAPA (Brown et al, BMC Pediatric 2010). Since autoinflammatory diseases by definition are mediated by cells of the innate immune system, and the major innate immune cell, the neutrophil, is abundant in the circulation during PFAPA episodes we decided to investigate neutrophil function during the febrile and afebrile state in the same cohort.

PW03-003 – Altered neutrophil function in PFAPA