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.
Recurrent episodes of fever are accompanied by local inflammation. They include two autosomal dominant disorders: CAPS (Cryopyrin Associated Periodic Syndromes) and TRAPS (TNFR-Associated Periodic Syndrome) and autosomal recessive ones: FMF (Familiar Mediterranean Fever) and MAPS (Mevalonate kinase Associated Periodic fever Syndrome).
PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis a Adenitis) syndrome is an idiopathic disease with unknown aetiology..