Abstract
Periodic fever syndromes are clinical entities classified as autoinflammatory diseases. A relatively new group of periodic fever syndromes are characterised by failure of innate immunity mechanisms.
Recurrnet episodes of fever are accompanied by local inflammation. They include two autosomal dominant disorders: CAPS and TRAPS and autosomal recessive ones: FMF and MAPS.
PFAPA syndrome is an idiopathic disease with unknown aetiology.