The First International Conference on Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome

In the last 20 years, autoinflammatory syndromes, a new category of immune-mediated diseases, have been described. These diseases are defined as attacks of inflammation, often multisystem, that are unprovoked (or triggered by a minor event) and primarily are related to dysregulation of the innate immune system.1 Many of the syndromes are monogenically inherited. Unlike autoimmune diseases, there is a relative deficiency of both autoantibodies and autoreactive T lymphocytes. The inflammatory response is usually mediated by proinflammatory cytokines, especially interleukin (IL)-1 secreted by granulocytes and monocytes.

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