Editorial: Autoinflammatory diseases

The term ‘periodic disease’ was first used in the medical literature in 1948 for the condition later named as ‘familial Mediterranean fever’ (FMF). Since then many different genetically determined diseases characterized by recurrent fever were collectively termed as ‘hereditary periodic fevers’ (HPFs). The spectrum of such diseases expanded as the underlying molecular mechanisms and genetic defects were revealed. Kastner first coined the term “autoinflammatory syndromes” in 1999 for such group of disorders that did not fit into the classical immunemediated diseases. These, mainly monogenic, conditions share distinct clinicopathologic features and are characterized by recurrent episodes of inflammation, without high-titer autoantibodies or antigen-specific T cells. They are quite rare diseases with no gender predilection. The onset is usually before ten years of age.